tag:blogger.com,1999:blog-79616558010185314682024-03-14T04:01:39.466-04:00Garrett's Houseto promote awareness and offer education about a rare genetic skin condition called Epidermolysis Bullosa. Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.comBlogger176125tag:blogger.com,1999:blog-7961655801018531468.post-37406199968860103712024-02-29T20:59:00.000-05:002024-02-29T20:59:19.385-05:00Personal Stories: Piper Rose<p> <span style="font-family: Georgia, serif; font-size: 12pt; white-space-collapse: preserve;">🌹Piper Rose, my Leap Year Baby🌹</span></p><span id="docs-internal-guid-b1ad4aa0-7fff-8846-3540-ccb26e0bd5b0"><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">My daughter Piper was born via c-section at 39 weeks right at the start of the COVID madness. 2/29/2020 at 2:29pm I had a normal and healthy pregnancy, but her birth was anything but normal. When the doctor pulled her out of my womb, her lower body was completely raw. The hospital staff was completely shocked and horrified. No one had ever seen anything like it. As they attempted to clear her airway, blood started pouring out. She was whisked away straight to the NICU before I could even see her. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The doctor stitched me up as quickly as he could as I laid there sobbing. I was alone in the delivery room, and had no idea what was going on with my baby girl. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Once I went to recovery, all the nurses were quiet around me, none one would tell me how my baby was doing; I was terrified she was dead, </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Eventually, the neonatologist came into recovery to speak to me. A nurse came with him as well. I was sitting there with tears streaming down my face, afraid of the news I was about to receive. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">They had good news and bad news. The good: Piper was alive and they think they knew what was wrong. The nurse had seen it before, but only once in her 25 years career. The bad: they thought Piper had a rare skin condition called Epidermolysis Bullosa or EB for short. I had never heard of it before, but it sounded very scary. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The doctor explained she had very fragile skin and would need to be wrapped in protective bandages to prevent infection. I asked if Piper would get better; the doctor said he didn’t know. Some get better, some get worse, some die. And worst of all, there was no cure :( </span></p><br /><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">They said I could see her as soon as I felt up to moving. I just laid there in shock at the news I just received. I was full of so many emotions. I was all alone and had no idea if my baby would live or die. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">A few hours later I felt I could move into a wheel chair and a nurse wheeled me up to the NICU. There was my baby, in this incubator covered in bandages and wires. But she was beautiful with her Fuzzy blonde hair and blue eyes. She was a whopping 9 pounds, 9 oz and was 21 inches long. Aside from the skin issue, she was perfectly healthy. They wouldn’t let me hold her just let, so I sat there rubbing her head telling her how much I loved her. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The next few days, there were so many ups and downs. They sent off blood for genetic testing to confirm her diagnosis. The doctors began researching EB to learn how to best care for Piper. I had also enlisted my best friend, Ava, to research EB. I couldn’t bring myself to look on-line for fear of what I might learn. Ava found so much on line about EB; some good, some bad, some terrifying. She got us a list of the correct bandages and ointments to use, links to videos on how to bandage an EB baby, the best clothes to buy, diapers to wear and bottles to use. She found support groups online with so many helpful people also going through the same thing. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Over the days I learned how to carefully bandage my baby to protect her skin from getting worse or getting an infection. She struggled to breastfeed, so she was fed with a special bottle. At first she started out eating amazing, but slowly started to struggle when she was around 10 days old, her breathing began to get “noisy” but no one knew why. Around that time, her doctor learned about some hospitals that had an EB clinic. He contacted one of them and spoke to the dermatologist there. She had dealt with EB for many, many years. She gave the doctors some good tips and suggestions but feared Piper had the worst form of EB, Junctional Hertliz, based on his description of her. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">A couple of days later Piper stopped eating altogether and a feeding tube had to be placed down her nose into her stomach. It was so hard to watch my baby fight for her life. I felt she was fading right before my eyes. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">When she was 2 weeks old, we still had no answers. The unknown of COVID was delaying everything. The doctor felt she might receive better care at a hospital with an EB clinic. I was all for anything to help my baby get better. He contacted the nearest hospital with an BB clinic, but because of the uncertainty of COVID, they weren’t accepting transfers. The dermatologist however, offered to watch a bandage change via video conference before deciding to override the transfer request.</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The next day, everything was set up and the doctor at the EB clinic watched me carefully unwrap, clean and rewrap my fragile baby. Afterwards, we had a meeting and the dermatologist felt her hospital couldn’t provide any better care than Piper was already receiving. She said transferring her would be risky and she might not survive the transfer. She also said that in her many years of experience of treating EB patients, she felt Piper had the Junctional Hertliz form of EB, and she likely wouldn’t live past four months. I asked about having a feeding tube placed, only to be told she might not survive the surgery. It felt like no matter what we did, she was going to die anyway. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">That was devastating news to hear. The thought of my baby girl dying was more than I could bear. I spent the next few days holding my baby girl, trying to decide what was best for her. I wanted her to live, but what kind of life would she have??? Would she be in pain everyday of her life?. Would she ever walk? Eat without a feeding tube? Here I was a brand new parent, all alone holding my fragile baby girl, feeling totally lost. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Over the next few days, Piper started to decline. Blood work showed she was anemic and needed a blood transfusion. Two days later her breathing got worse and she spiked a fever; more blood work was done, and it was discovered she had a blood infection. Her doctor called the EB clinic for advice on how to best treat the infection, and the doctor asked to speak to me. She said I could choose to treat the infection, and Piper might survive, or I could decide to make her comfortable and let her go. It was an awful decision to have to make. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">I sat there in the NICU rocking my baby girl while trying to decide what to do. But I never got to make the decision; at 2:50am on a Thursday, while holding my baby in my arms, she looked up at me and smiled, closed her eyes and took her last breath. I waited too long to decide. She was 21 days old. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The autopsy revealed that her epiglottis was severely inflamed and it closed off the opening in the back of the throat that leads to the upper airway. There was evidence of silent reflux, mucus plugs and swelling in her upper airways. The NICU doctor didn’t know what any of that meant in terms of being related to EB or if it would have been treatable. I later learned what it all meant and And the what if’s still haunt me to this day.</span></p><br /><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The day of Piper’s funeral, her genetic results came back, but I couldn’t bring myself to open them. I put them in a safe place and tried to move on with my life. Opening them wouldn’t change anything and I worried the results would make me question every decision I had made in those 3 weeks. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">On Piper's first birthday, I decided to open the results. Much to my shock, Piper didn’t have Junctional Hertliz EB like the doctor at the EB clinic suspected. She didn’t even have Junctional EB. The results listed her as having generalized severe EB simplex. I was completely floored. I had no idea what any of this meant, so I began to research the hell out of EB and specifically, EB simplex. I couldn’t believe what I was reading. Most with EB simplex survived. I joined social media groups and read other people’s experiences. I researched her specific Keratin 5 mutation. I spent hours on-line looking for any information I could on her mutation, c. 1421c>t, trying to understand what it meant, and why it caused EB. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">I found another EB family with the same mutation. The mom has EB, and 3 of her kids were also born with EB. Sadly she too has an EB angel. Knowing it wasn’t just Piper, made me feel less alone, but it still sucked. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;"> I met with a genetics doctor to help understand how Piper got EB. She explained how one gets EB simplex, and recommended I be tested. It was discovered I didn’t have the mutation. I tracked down her “father” and demanded he be tested. He didn’t have the mutation either. The doctor explained that her EB was caused by a spontaneous mutation. A fluke of nature. I learned that silent reflux was common in EB and totally treatable. My baby could have lived. The more I researched, the more angry I got. I was so, so angry, why Piper?? Why me?? How could the doctor have been wrong? Why did I listen to her?? My baby should have lived. I was more angry at that point, than I had been anytime in the past year. I felt like I had failed my baby. I blindly trusted the doctors because that is what we are taught to do. But the doctors was wrong, so very, very wrong.</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">The genetics doctor explained the odds of having another baby with EB would be very, very small. But I didn’t want another baby, I wanted Piper. I wanted my baby in my arms.</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">This entire experience has opened my eyes to so much in this world. It has also caused me to trust doctors a lot less. I am currently going to nursing school to be a NICU nurse. I hope I never get an EB baby in my NICU, but if I ever do, I’ll give him or her the best care I can. Everything I do is for Piper. I don’t want her death to be in vain.</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;"> </span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Thank you to Garrett’s House for allowing me to share my story. I miss my baby every single day and I wake up everyday to keep her memory alive. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">❤</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Lynnea, Piper’s mommy.</span></p><div><span style="font-family: Georgia, serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;"><br /></span></div></span>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-39960214482870506542024-01-09T18:53:00.005-05:002024-01-09T18:53:47.201-05:00Updated Recommended Blood Work for those with EB<p><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-weight: 700; white-space-collapse: preserve;">**New Release January 2024**</span></p><p><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-weight: 700; white-space-collapse: preserve;">Recommend Blood Work for All Forms of EB starting at age 1 OR for those who have never had blood work done before</span></p><span id="docs-internal-guid-a4de039d-7fff-29b2-c660-16b06fc9acce"><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">TSH</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">T4 Free</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Vit D3</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">CRP</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Sed Rate</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">CMP (comprehensive metabolic which includes liver and kidney functions)</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">CBC w/ Differential </span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Vit C</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">B12</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Zinc</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; font-weight: 700; vertical-align: baseline; white-space-collapse: preserve;">Other Yearly Tests that are Recommended for all forms of EB</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">EKG </span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Echocardiogram</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Dexa Scan</span></p><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; font-weight: 700; vertical-align: baseline; white-space-collapse: preserve;">Infants with Junctional EB should have the following done starting at age 1 month due to malnutrition issues and months and as needed going forward:</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">CRP</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Sed Rate</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">CMP (comprehensive metabolic which includes liver and kidney functions)</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">CBC w/ Differential </span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Vit C</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Zinc</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Vit D3</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; font-weight: 700; vertical-align: baseline; white-space-collapse: preserve;">Additional recommended test for those with RDEB, JEB or severe EBS and/or have a history of anemia, liver and/or renal issues</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Ammonia</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Full Iron Panel</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Copper</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;">Expanded Hepatic Panel</span></p><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 12pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; font-weight: 700; vertical-align: baseline; white-space-collapse: preserve;">EB knowledgeable nutritionists are recommending the following nutrients, micronutrients and heavy metal panels at least once every 1-3 years and then repeat as needed based on previous results, patients nutritional status and overall health. </span></p><br /><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin A</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">B Vitamins</span></p></li><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Thiamin – Vitamin B1</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Riboflavin – Vitamin B2</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Niacin – Vitamin B3</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Pantothenic Acid – Vitamin B5</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin B6</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Biotin – Vitamin B7</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Folate (Folic Acid)</span></p></li><li aria-level="2" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin B12</span></p></li></ul><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin C</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin D</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin E</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Vitamin K</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Choline</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Calcium</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Chloride</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Chromium</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Copper</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Fluoride</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Iodine</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Iron</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Magnesium</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Manganese</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Molybdenum</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Nickel</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Phosphorus</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Potassium</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Selenium</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Sodium</span></p></li><li aria-level="1" dir="ltr" style="color: #484c58; font-family: Verdana, sans-serif; font-size: 11.5pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="color: black; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; text-wrap: wrap; vertical-align: baseline;">Zinc</span></p></li></ul><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;"> </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Verdana, sans-serif; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; font-weight: 700; vertical-align: baseline; white-space-collapse: preserve;">Heavy Metals:</span><span style="font-family: Verdana, sans-serif; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;"> Arsenic, Cadmium, Cobalt, Lead, Mercury </span></p><div><span style="font-family: Verdana, sans-serif; font-size: 11pt; font-variant-alternates: normal; font-variant-east-asian: normal; font-variant-numeric: normal; font-variant-position: normal; vertical-align: baseline; white-space-collapse: preserve;"><br /></span></div></span>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-52748932328360523842023-02-06T14:08:00.002-05:002023-02-06T17:45:50.556-05:00Back to Blended Diet Feeds<p><span style="font-family: helvetica; font-size: medium;">Due to a side effect of a medication, we have gone from 90% oral feeds and 10% g-tube feeds, to 90% g-tube feeds and 10% oral feeds.</span></p><p><span style="font-family: helvetica; font-size: medium;">Because he is much older now, his nutritionist helped me determine the amount of calories, vitamins and nutrients he needs daily to heal, gain and grow. </span></p><p><span style="font-family: helvetica; font-size: medium;">Though physical activities levels vary from person to person, those with EB tend to have very high <span style="background-color: white; color: #202124;">Metabolism because the body is in a constant stay of healing and regenerating skin. That requires so much energy, calories and nutrition. </span></span></p><p><span style="color: #202124; font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="color: #202124; font-family: helvetica; font-size: medium;">This is the calculator the nutritionist used for us: <a href="https://www.nal.usda.gov/human-nutrition-and-food-safety/dri-calculator" target="_blank">DRI Calculator </a></span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">While Joey is a very active child, she recommended choosing the very active option from kids with EB since there is no specific calculator for those with EB. </span></p><p><span style="font-family: helvetica; font-size: medium;">Here is the recommendation for a pre-teen male with generalized severe EB Simplex. For the Vitamin, Mineral and Nutrients, she recommended making sure he gets at least the minimum amounts thru food but no more than the maximum amounts per day. We will do blood work in 6 months to see if these recommendations are working for him. </span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">**Again, please check with your child's doctor before changing their diet, because what is best for one child, isn't best for all kids with EB.** </span></p><p><br /></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: verdana; font-size: medium;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEifZk_y6zFCEOL2-aJuQ6qidBmpE4i78c0AzjPmIeVwhCiMupvFQyav2pZYzxbf-C9SBUfE8JF2NUwe1y1rzr5ZzI1KghKWh7uaseD1ZwhWljrztGp-3BVmvszNFtg8ZGYy-WwyU_PVKwIyTKrIjjHQu4qdBlt2eIZFxwKc6h3tKW3Z7zjhxVqrndff/s487/Screenshot%202023-02-06%201.45.00%20PM.png" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="487" data-original-width="440" height="320" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEifZk_y6zFCEOL2-aJuQ6qidBmpE4i78c0AzjPmIeVwhCiMupvFQyav2pZYzxbf-C9SBUfE8JF2NUwe1y1rzr5ZzI1KghKWh7uaseD1ZwhWljrztGp-3BVmvszNFtg8ZGYy-WwyU_PVKwIyTKrIjjHQu4qdBlt2eIZFxwKc6h3tKW3Z7zjhxVqrndff/s320/Screenshot%202023-02-06%201.45.00%20PM.png" width="289" /></a></span></div><span style="font-family: verdana; font-size: medium;"><br /></span><p></p><div><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjP6jD0FmM6B4azY6rtthshcOPF4td0OeLh_tgslGkHeys4Zbwcdg6BlfiH7e8BGhD9Z0iA0mOTOK8RfHOn_5PH9NjHG79Tk4NlqYbryzRYzSZlSN96VilSVXDt0gzWKhVgPVDnVHfcODrYrjyZ8k0bvya9MnvDxsFNrL62I6p3dR2LGrXLD-vZe_Yi/s755/Screenshot%202023-02-06%201.45.26%20PM.png" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="517" data-original-width="755" height="304" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjP6jD0FmM6B4azY6rtthshcOPF4td0OeLh_tgslGkHeys4Zbwcdg6BlfiH7e8BGhD9Z0iA0mOTOK8RfHOn_5PH9NjHG79Tk4NlqYbryzRYzSZlSN96VilSVXDt0gzWKhVgPVDnVHfcODrYrjyZ8k0bvya9MnvDxsFNrL62I6p3dR2LGrXLD-vZe_Yi/w444-h304/Screenshot%202023-02-06%201.45.26%20PM.png" width="444" /></a></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjnduEcBVorUTjfQfMQkoPFsKlTbRkKbKynEnPWfciAAIi1hubFdZm7VeyHKr35q7UDEkbYP7NGk_JEa1NE8WKhimbtWUKnSbP1tlEGsN_tFUpFL-SdbFibQabY4dEkr33yUPZlvu5kcgVZ37qnhN2Os-jJnUkXevaeAWTbnJjYsKEJmwyBYScbmjIX/s638/Screenshot%202023-02-06%201.45.55%20PM.png" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="556" data-original-width="638" height="385" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjnduEcBVorUTjfQfMQkoPFsKlTbRkKbKynEnPWfciAAIi1hubFdZm7VeyHKr35q7UDEkbYP7NGk_JEa1NE8WKhimbtWUKnSbP1tlEGsN_tFUpFL-SdbFibQabY4dEkr33yUPZlvu5kcgVZ37qnhN2Os-jJnUkXevaeAWTbnJjYsKEJmwyBYScbmjIX/w441-h385/Screenshot%202023-02-06%201.45.55%20PM.png" width="441" /></a></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgWwEx8QC42dntUL_rj1IXcIJTtLCAdUXN0OuaW4pSiD0_gNGtpI2jPqb8Dxy7CWqC0lynIg4V9KPU828V_SVvIZ1FRZtTWGYtHj9d531R1OCPsJjsiErD3ru-BIlHpsccbogjRb6PunsUj5kDwZEPRSK0tK3RRdRwUBRH_VTULao7KKwGQwBEWoGcO/s571/Screenshot%202023-02-06%201.46.33%20PM.png" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="571" data-original-width="533" height="437" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgWwEx8QC42dntUL_rj1IXcIJTtLCAdUXN0OuaW4pSiD0_gNGtpI2jPqb8Dxy7CWqC0lynIg4V9KPU828V_SVvIZ1FRZtTWGYtHj9d531R1OCPsJjsiErD3ru-BIlHpsccbogjRb6PunsUj5kDwZEPRSK0tK3RRdRwUBRH_VTULao7KKwGQwBEWoGcO/w408-h437/Screenshot%202023-02-06%201.46.33%20PM.png" width="408" /></a></div><div><br /></div><div><span style="font-family: verdana; font-size: large;"><br /></span></div><div><span style="font-family: verdana; font-size: large;">The nutritionist also sent me the following about what is most important in wound healing from a nutritional standpoint. </span></div><div><b><br /></b></div><div><span id="docs-internal-guid-559dfef6-7fff-01ea-182e-1c0837c68b76"><span style="font-size: medium;"><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><br /></p><span style="font-family: verdana;"><br /></span><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: verdana;"><b>Vitamin D</b>: In recent studies have identified Vitamin D receptors in a variety of tissues where their presence was previously unknown. Cathelicidin, an antimicrobial peptide induced by vitamin D, promotes wound healing. These researchers concluded that vitamin D and its receptor signaling regulates structural integrity and transport functions of epithelial barriers with implications for wound healing.</span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><br /></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><b>Protein:</b> Protein’s most important use is production of collagen that serves as scaffolding for scar in healed wounds. The proteins are also necessary for forming new blood vessels and encouraging healing cells to migrate to the wound surface. </span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><br /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><b>Amino acids:</b> Protein is made of amnio acids. Among amino-acids, those that play an important role in wound healing, are arginine and glutamine. Arginine is essential for the inflammatory process and synthesis of collagen. Arginine also stimulates the immune system to begin the healing process. Glutamine plays several roles via its metabolic, enzymatic, antioxidant, and immune properties. In wounds, it protects against the risk of infections by stimulating production of immune fighting proteins. Glutamine is also a precursor of glutathione—an antioxidant and an essential cofactor in stabilizing cells. The recommendation is to have some arginine and glutamine included in the protein supplements.</span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><br /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: verdana;"><span style="background-color: white; white-space: pre-wrap;"><b>Vitamin C</b>: </span><span style="background-color: white; white-space: pre-wrap;">Vitamin C, also known as ascorbic acid (AA), is involved in all phases of wound healing. In the inflammatory phase it is required for neutrophil apoptosis and clearance. During the proliferative phase, AA contributes towards synthesis, maturation, secretion and degradation of collagen. The role of vitamin C in wound healing is believed to be due to an influence on collagen formation, immunomodulation, and antioxidant functions. Deficiency results in impaired immune response during the inflammatory phase with increased capillary fragility and reduced collagen tensile strength and synthesis during the proliferative and remodeling phases,</span><span style="background-color: white; white-space: pre-wrap;">It also stimulates collagen and elastin formation. It is essential for stabilizing the structure of collagen with strong bonds and crosslinks to strengthen the healing tissue. Without this stabilization, the structure becomes fragile. Vitamin C also provides tensile strength to newly built collagen; otherwise, new tissue could not stretch without tearing. Tensile strength is important in pressure ulcer healing because healed pressure ulcers are susceptible to future skin breakdown. Vitamin C also is required for proper immune system function, a consideration in patients with open wounds. </span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><br /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: verdana;"><span style="background-color: white; white-space: pre-wrap;"><b>Zinc:</b> Zinc plays a major role in regulating every phase of the wound healing process; ranging from membrane repair, oxidative stress, coagulation, inflammation and immune defense, tissue re-epithelialization, angiogenesis, to fibrosis/scar formation. </span><span style="background-color: white; white-space: pre-wrap;">Decreased zinc levels result in decreased cytotoxicity of natural killer cells, impaired phagocytosis in macrophages and neutrophils, and decreased number of granulocytes. Also with zinc deficiency, B-cell precursors and mature B-cells are reduced. Zinc deficiency affects all phases of wound healing. In the inflammatory phase, there is decreased immunity and increased susceptibility to infections. In the proliferative phase, there is impaired collagen synthesis and tensile strength. Finally, in the remodeling phase, there is a dampening of fibroblast proliferation, collagen synthesis, and epithelialization, therefore </span><span style="background-color: white; white-space: pre-wrap;">the body needs adequate Zinc stores. </span><span style="background-color: white; white-space: pre-wrap;">However, excess zinc supplementation can interfere with the absorption of other cations, specifically iron and copper. Therefore supplementation should be based on individual need. </span></span></p><div><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><br /></span></span></div><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: verdana;"><br /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: verdana;"><b>Vitamin A</b>: It’s one of the most essential nutrients for wound healing since it helps “Control Inflammatory Response”. Vitamin A is required for epithelial and bone tissue development, cellular differentiation, and immune system function.Vitamin A is known for its ability to stimulate epithelial growth, fibroblasts, granulation tissue, angiogenesis, collagen synthesis, epithelialization, and fibroplasia. Vitamin A also has the unique ability to reverse the inhibitory effects of steroids on wound healing. Steroids reduce the ability of healing cells to migrate to the wound and build strong collagen bonds. Vitamin A can reverse this effect! In addition to its role in the inflammatory phase of wound healing, Vitamin A will also strengthen the collagen bonds and reduce the ability of wound inhibitory enzymes to counteract the healing process. </span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: verdana;"><br /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: verdana;"><span style="font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: verdana;"><b>Iron:</b> Iron was previously postulated as beneficial in wound healing, due to its status as a cofactor in collagen synthesis. In wound healing, iron deficiency results in impaired T cell and phagocyte function in the inflammatory phase and decreased tensile strength and collagen synthesis in the proliferative phase However, iron supplementation has been shown to prolong inflammation, and no evidence exists that iron supplementation benefits wound healing. Patients with iron deficiency anemia, which is known to inhibit wound healing due to decreased oxygen transport to proliferating tissues, may benefit from iron supplementation; however, more data is required to establish treatment guidelines.</span></span></p><div><br /></div><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="background-color: white; white-space: pre-wrap;"><span style="font-family: verdana;"><b>Blood sugars</b>: Though not a nutritional supplement, blood sugar control is essential for healing. Though glucose is the fuel for cells, when the blood stream is saturated with sugar, the healing cells become sluggish and stop healing. They also block the path for important nutrients and oxygen to pass into the wound. Unfortunately, bacteria use this excess sugar to wreak havoc on wounds. This leads to an infection with no healing cells able to fight the bacteria. We often see normally benign bacteria cause severe wounds due to elevated blood sugar.</span></span></p><p dir="ltr" style="background-color: white; font-family: helvetica; line-height: 1.76087; margin-bottom: 30pt; margin-top: 16pt;"><br /></p><p dir="ltr" style="background-color: white; font-family: helvetica; line-height: 1.76087; margin-bottom: 0pt; margin-left: 36pt; margin-top: 16pt; padding: 0pt 0pt 30pt;"><br /></p><p dir="ltr" style="background-color: white; font-family: helvetica; line-height: 1.76087; margin-bottom: 30pt; margin-left: 36pt; margin-top: 0pt;"><br /></p><br /><p dir="ltr" style="background-color: white; font-family: helvetica; line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt; padding: 0pt 0pt 11pt;"><br /></p><br /></span></span></div><span style="font-family: verdana; font-size: medium;"><br /></span></div>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-48101400885302036832023-01-26T19:41:00.003-05:002023-01-26T19:41:56.643-05:00Rachel and Hon <p><span style="font-family: helvetica; font-size: medium;">Today we have a guest blogger with us! Rachel is here to share a story about her husband Hon❤</span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhTW5cl6xl6zEY5S_V6X3CpzNBruRHxcKHzZof2S2fL84RomPwpzYtRaEE0GOGid5sl73f0pIJiqwVpBviRPHQKN8wN07OON-AzRz7yjtf5yMo9cufK6USjDFVpJ8rbrBbxW_2ZWQMHtc_rl3YKibEBUCAybcAemckXacgC-QtKLaAj43ozmht8CMxo/s1440/Hon-Rach-A.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: helvetica; font-size: medium;"><img border="0" data-original-height="1078" data-original-width="1440" height="240" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhTW5cl6xl6zEY5S_V6X3CpzNBruRHxcKHzZof2S2fL84RomPwpzYtRaEE0GOGid5sl73f0pIJiqwVpBviRPHQKN8wN07OON-AzRz7yjtf5yMo9cufK6USjDFVpJ8rbrBbxW_2ZWQMHtc_rl3YKibEBUCAybcAemckXacgC-QtKLaAj43ozmht8CMxo/s320/Hon-Rach-A.jpg" width="320" /></span></a></div><span style="font-family: helvetica; font-size: medium;"><br /></span><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEinwgGowqdooMhrnke4_K4jPVuGJcecDjWb25RWl2vid5waonh0lBnnCAt914IQDO-ZT2ZHbjqIMF3orzKwrDgM-6Fja_q4K2MJcLTkQzsIosorVwAl3D_yxnbvRwCSfwuyrXXTsxfUQhlqsNN9KdL4joOg5asXCZ2XDwboaQhHbj0ZNkUXwpKzFCAT/s640/san-diego-hubbie.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: helvetica; font-size: medium;"><img border="0" data-original-height="480" data-original-width="640" height="240" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEinwgGowqdooMhrnke4_K4jPVuGJcecDjWb25RWl2vid5waonh0lBnnCAt914IQDO-ZT2ZHbjqIMF3orzKwrDgM-6Fja_q4K2MJcLTkQzsIosorVwAl3D_yxnbvRwCSfwuyrXXTsxfUQhlqsNN9KdL4joOg5asXCZ2XDwboaQhHbj0ZNkUXwpKzFCAT/s320/san-diego-hubbie.jpg" width="320" /></span></a></div><span style="font-family: helvetica; font-size: medium;"><br /></span><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;"><b>I fell in love with a Butterfly…</b></span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">I met Hon back in 2009. He was busy working for whizz kids, teaching children how to use their wheelchairs. We immediately hit it off and decided to go on a date to Monkey World, Hon always jokes I took him to see monkey butt! Hehe! We fell in love. We got married a year later and the rest is history…</span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">We travelled and went to new places as often as we could. Hon has such a zest for life, nothing stops him, certainly not EB. Before we met, Hon went to University to get a Degree. Hon is highly intelligent and the bravest person I know. Hon also did a Skydive in 2005, he really is fearless!</span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">Life with Hon is never boring, especially with EB, it certainly makes life unpredictable! Hon has Junctional Non- Herlitz form of EB since birth. Junctional is one of the rarer forms of EB. There is no cure. Hon has used a wheelchair for decades due to the chronic blistering and open wounds on his feet, they are raw. Hon has open wounds head to toe. </span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">We spend hours and hours doing baths/ dressings/ creams/ popping blisters daily. EB is 24/7, 365 days of the year, it is relentless. Hon is at a very high risk of infection and Sepsis due to the amount of open wounds and the immunosuppressant’s he has to take.</span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">Hons attitude is always you have to control EB, otherwise it will control you… </span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="font-family: helvetica; font-size: medium;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;">Hons Kidneys deteriorated in 2010 we hit Dialysis with a bang. I quickly learnt how to do haemodialysis, so we could do it at home. 2 years on, we had a miracle donor and the Worlds 1</span><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="vertical-align: super;">st</span></span><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"> Kidney Transplant with EB in 2012. 10 years on, still going strong. </span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">Due to the Kidney Transplant, Hon has to take several immunosuppressant’s, this has clipped our wings during Covid times, as Hon is clinically extremely vulnerable, so we shield as protection. The immunosuppressant’s have caused 2 episodes of Squamous Cell Carcinoma last year, leaving Hon with extremely large/ deep surgical wounds to tricky areas, along with the usual EB wounds. </span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">For me the hardest thing to watch is the Corneal Abrasions. They are spontaneous, happen when you least expect it. Hon describes it as though someone throws acid in his eyes. The pain must be excruciating. All we can do is keep our eyes closed, in a darkened room, with eye drops. Sometimes its days, sometimes weeks. The worst was 2 months, to be rendered temporarily blind and not able to open his eyes is heart-breaking. I always feel so helpless, as there is nothing I can do except be there and love him. </span></span></p><p dir="ltr" style="line-height: 1.295; margin-bottom: 8pt; margin-top: 0pt;"><span style="background-color: transparent; color: black; font-style: normal; font-variant: normal; font-weight: 400; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;"><span style="font-family: helvetica; font-size: medium;">We are very lucky, we have each other and we battle EB together, we are a team. Hon is truly amazing, he is my soulmate, my forever love and my Butterfly x</span></span></p><p><span id="docs-internal-guid-de7baf99-7fff-90c2-9f39-fc0fa3eff719"><br /></span></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-20103335143599048802022-10-31T20:26:00.005-04:002022-10-31T20:26:58.040-04:0020 years of EB Stats <p><span style="font-family: helvetica; font-size: medium;">Normally I would go in great depth and detail when it comes to EB statistics, however, I have had many of my ideas and posts "taken" from my blog without permission and passed off as the work of someone else. So I am hesitant to share too much detail for fear it'll be "stolen" as well.</span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">Between the years 2001 and 2021, I came across 2,018 new EB babies born during that time frame. (world wide)</span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">1,003 cases were boys</span></p><p><span style="font-family: helvetica; font-size: medium;">987 cases were girls</span></p><p><span style="font-family: helvetica; font-size: medium;">in 28 cases the gender was not known</span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">The most common month to be born in was July. In the US, Texas had the most cases of EB babies.</span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">32% (650 babies) were born with some form of Junctional EB</span></p><p><span style="font-family: helvetica; font-size: medium;">33% (663 </span><span style="font-family: helvetica; font-size: large;">babies</span><span style="font-family: helvetica; font-size: large;">) were born with some form of EB Simplex</span></p><p><span style="font-family: helvetica; font-size: medium;">31% (623 </span><span style="font-family: helvetica; font-size: large;">babies</span><span style="font-family: helvetica; font-size: large;">) were born with some form of Dystrophic EB</span></p><p><span style="font-family: helvetica; font-size: medium;">less than 1% (3 </span><span style="font-family: helvetica; font-size: large;">babies</span><span style="font-family: helvetica; font-size: large;">) were born with Kindler Syndrome </span></p><p><span style="font-family: helvetica; font-size: medium;">4% (82 </span><span style="font-family: helvetica; font-size: large;">babies</span><span style="font-family: helvetica; font-size: large;">) had an unknown form of EB</span></p><p><span style="font-family: helvetica; font-size: medium;"><br /></span></p><p><span style="font-family: helvetica; font-size: medium;">The mortality rate for that period of time was 19% (390 </span><span style="font-family: helvetica; font-size: large;">babies</span><span style="font-family: helvetica; font-size: large;">) </span></p><p><br /></p><p><br /></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-22271555783993590602022-01-31T18:38:00.000-05:002022-01-31T22:58:18.163-05:00Our arsenal of ointments in wound care<p><span style="font-family: helvetica; font-size: large;">Aquaphor is probably the #1 ointment used among many with EB. We don't use Aquaphor for a variety of reasons. And because of that I get asked a lot what ointment(s) we use. And my answer is this "depends on the wound"</span></p><p><span style="font-family: helvetica; font-size: large;">Between my 4+ decades living with EB and trying different things with the kids, we have tried sooooo many different ointments, lotions, oils, etc...</span></p><p><span style="font-family: helvetica; font-size: large;"><br /></span></p><p><span style="font-family: helvetica; font-size: large;">Below are the ointments we use the most. I'll also explain what type of wound we use each on. </span></p><p><span style="font-family: helvetica; font-size: large;"><br /></span></p><p style="text-align: center;"><span style="font-family: helvetica; font-size: x-large;"><b>** DISCLAIMER**</b></span></p><p><span style="font-family: helvetica; font-size: large;">The following is NOT medical advice. As always check, with your medical professional before trying something new on yourself/your child. Some of the ointments I will talk about are NOT safe for infants or young child, but I'll make a note of those.</span></p><p><span style="font-family: helvetica; font-size: large;"><br /></span></p><p><span style="font-family: helvetica; font-size: large;">Coconut Oil: We use organic, unrefined coconut oil for places that are delicate, the face, near the mouth, eye, ears. Coconut oil is safe for all ages and types of EB. Here is one <a href="https://www.amazon.com/Parachute-Naturalz-Organic-Virgin-Coconut/dp/B0762F5X6B/ref=sr_1_1_sspa?crid=7YY0E6PNADGS&keywords=coconut+oil&qid=1643649775&sprefix=cocnut+oi%2Caps%2C166&sr=8-1-spons&psc=1&smid=A1OBRPXY3FS42G&spLa=ZW5jcnlwdGVkUXVhbGlmaWVyPUEyOVRVSzQ2MDZUU0FLJmVuY3J5cHRlZElkPUEwODk0OTU5MVhCOVY3MjNOQ1NLRSZlbmNyeXB0ZWRBZElkPUEwMzA5MjA3N0FKOVdONVZLSDUwJndpZGdldE5hbWU9c3BfYXRmJmFjdGlvbj1jbGlja1JlZGlyZWN0JmRvTm90TG9nQ2xpY2s9dHJ1ZQ==" target="_blank">brand</a> we have used before.</span></p><p><span style="font-family: helvetica; font-size: large;"><br /></span></p><p><span style="font-family: helvetica; font-size: large;">In place of Aquaphor, I like <a href="https://www.amazon.com/Burts-Bees-Baby-Multipurpose-Ointment/dp/B006YTX0HQ/ref=sr_1_2?keywords=burts+bee+ointment+multipurpose&qid=1643649974&sprefix=burts+bee+oint%2Caps%2C147&sr=8-2" target="_blank">Burt's Bee Multipurpose Ointment</a>. It is "healthier" than traditional Aquaphor, but not as cost efficient for those who need large quantities. It has been good for dry skin or dry woulds or just places that need extra moisture. This ointment is safe for all ages as well.</span></p><p></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEigeKcHY6n_mOJ3rfqhfNrn4T96vzte5Sv8rzDS6dTGlKLz7huv_iBmJen0T4eFzM54DLIoUBRoPrXzshDHv5xVZhHNetbnja8TR9JvUmb6nJfWfzac15fhmlqkt4ZRKwjrsIJ5kQa7DT5wNfXMzGKoFd5sBh2KaXSONpzVkPd8rLsZHXBiYaZzD0xO=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="3456" data-original-width="4608" height="240" src="https://blogger.googleusercontent.com/img/a/AVvXsEigeKcHY6n_mOJ3rfqhfNrn4T96vzte5Sv8rzDS6dTGlKLz7huv_iBmJen0T4eFzM54DLIoUBRoPrXzshDHv5xVZhHNetbnja8TR9JvUmb6nJfWfzac15fhmlqkt4ZRKwjrsIJ5kQa7DT5wNfXMzGKoFd5sBh2KaXSONpzVkPd8rLsZHXBiYaZzD0xO=s320" width="320" /></a></div><br /><span style="font-family: helvetica; font-size: large;"><br /></span><p></p><p><span style="font-family: helvetica; font-size: large;">For use around G-tubes sites and the wounds in the diaper area we use <a href="https://www.amazon.com/Medline-Olivamine-Protectant-incontinence-dermatitis/dp/B078TLM8X3/ref=sr_1_4?keywords=remedy+ointment&qid=1643650271&sprefix=rememdy+oin%2Caps%2C157&sr=8-4" style="background-color: white;" target="_blank">Medline Remedy</a><span style="background-color: white; color: #0f1111;"> Olivamine Calazime Skin Protectant Paste Cream. It provides a very soothing cooling feeling, good for protecting again wetness, and granulation tissue. It was not around when my kids were babies, but if it had been, I would have used it. But definitely check with your doctor before using this.</span></span></p><p><span style="background-color: white; color: #0f1111;"></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEhZtBo9ahHFeAauboS8gnEUM-EkYgT9E6UT7t8fwhoP5YR7_3rF_oELq8ZWpAn_nrl2qrvfaa_igfWFznXGoaCITKL2bsy4nYmiyJOa6LTzSH2R0r87kC-gFLAS7V2pf13YTMNTNwQweCpskAeIZK9FHlgb7LOMNxqVmQnMU9pzzF-fsiqxAqp5Jif7=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="320" src="https://blogger.googleusercontent.com/img/a/AVvXsEhZtBo9ahHFeAauboS8gnEUM-EkYgT9E6UT7t8fwhoP5YR7_3rF_oELq8ZWpAn_nrl2qrvfaa_igfWFznXGoaCITKL2bsy4nYmiyJOa6LTzSH2R0r87kC-gFLAS7V2pf13YTMNTNwQweCpskAeIZK9FHlgb7LOMNxqVmQnMU9pzzF-fsiqxAqp5Jif7=s320" width="240" /></a></div><br /><span style="font-family: helvetica; font-size: large;"><br /></span><p></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">For intact, but dry and itchy skin, we like the following three creams. <a href="https://www.amazon.com/Aveeno-Emollients-Fragrance-Free-Moisturizing-Extra-Dry/dp/B0067H6G26/ref=sr_1_3?crid=U9T1TLNC31A1&keywords=aveeno+dry+skin+cream&qid=1643650569&sprefix=aveeno+dry+skin%2Caps%2C146&sr=8-3" target="_blank">Aveeno Dry Skin Cream</a> and <a href="https://www.amazon.com/Eucerin-Calming-Natural-Oatmeal-Enriched/dp/B000YGDRIW/ref=sr_1_1_sspa?crid=2G7DOLMQ3LHLQ&keywords=eucerin+skin+calming+cream&qid=1643650640&sprefix=eucerin++skin+%2Caps%2C152&sr=8-1-spons&psc=1&spLa=ZW5jcnlwdGVkUXVhbGlmaWVyPUExNkkyNlg0SEZCVDZOJmVuY3J5cHRlZElkPUEwMzE4ODU2MzBaOUUyRFFaUVFYWCZlbmNyeXB0ZWRBZElkPUEwNjExNTc2M0ozM0NKTlJYVFpXOCZ3aWRnZXROYW1lPXNwX2F0ZiZhY3Rpb249Y2xpY2tSZWRpcmVjdCZkb05vdExvZ0NsaWNrPXRydWU=" target="_blank">Eucerin Skin Calming</a> Cream and <a href="https://www.amazon.com/Coloplast-Sween-24-Moisturizing-Normal/dp/B01J8CVRZ2/ref=sr_1_1_sspa?keywords=sween+cream&qid=1643653379&sprefix=sween%2Caps%2C136&sr=8-1-spons&psc=1&smid=AFF8XSNGT0QQC&spLa=ZW5jcnlwdGVkUXVhbGlmaWVyPUExNFc0OUc2TlBXQjdVJmVuY3J5cHRlZElkPUEwMDgxMzA3MThLOVc0TkVHS1dEQyZlbmNyeXB0ZWRBZElkPUEwODczNDE2MThHNVJaUzNaV0MxMSZ3aWRnZXROYW1lPXNwX2F0ZiZhY3Rpb249Y2xpY2tSZWRpcmVjdCZkb05vdExvZ0NsaWNrPXRydWU=" target="_blank">Sween Cream</a>. I wouldn't use either on any open wounds or broken skin, but we find temporary relief for dry and itchy skin, especially when applied to the skin right after a bath or shower. </span></span></p><p><span style="background-color: white; color: #0f1111;"></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEiQb7NYw1ebzDP-N9WGXPpVSxEC2egJ5zFpmax1Hp_lc0_3Ii8QQQ0Ni9LQo2gt1bQaoe838XHJnTBLLQV6gZDGMeoFY1ssIDpgQBXppaS2KcjPhxpKlEXrvuCXYqB1cnuPbVqYLCrLzRn1mrvccRvAUkPeoAZ7Cxc4ObwVItLvzMEn3lpaW-1TbnUE=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="264" src="https://blogger.googleusercontent.com/img/a/AVvXsEiQb7NYw1ebzDP-N9WGXPpVSxEC2egJ5zFpmax1Hp_lc0_3Ii8QQQ0Ni9LQo2gt1bQaoe838XHJnTBLLQV6gZDGMeoFY1ssIDpgQBXppaS2KcjPhxpKlEXrvuCXYqB1cnuPbVqYLCrLzRn1mrvccRvAUkPeoAZ7Cxc4ObwVItLvzMEn3lpaW-1TbnUE=w198-h264" width="198" /></a><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEj0R0CpfhZ_qZ3_TAUhYcJJhVLoNb2Vub5_DJlTpye97k1EbSBWbT1DIL1YU9eCmGSikmXBg2qgN5bcrGgG4ClkNITHSp5cp7ceY7hgspWzycEgnVC-681AXXdJ_UtXVJYdFScN6Cc_HBvnG27LrQZWDxcaPfzq4Tdujq1L45OmW-JLxSu4Qz9WzwGz=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="320" src="https://blogger.googleusercontent.com/img/a/AVvXsEj0R0CpfhZ_qZ3_TAUhYcJJhVLoNb2Vub5_DJlTpye97k1EbSBWbT1DIL1YU9eCmGSikmXBg2qgN5bcrGgG4ClkNITHSp5cp7ceY7hgspWzycEgnVC-681AXXdJ_UtXVJYdFScN6Cc_HBvnG27LrQZWDxcaPfzq4Tdujq1L45OmW-JLxSu4Qz9WzwGz=s320" width="240" /></a></div><br /></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEi0RNwo4uuNx4bcX3GVwz_00e11laD205bfuqrXuf7TnLwRzzGcmtbXiSUYokJ1-zAWvVcdN7SpRLkKz9em_-c8enpqd2COKwzNKLPLz9elinFyj9XIu1w7Wf78_9nz4VQ2CGZwGGKBCIv2qJltoT4NyzX6XjyBYmEA27x91PTu0XJVn0VZeMRclPEK=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="320" src="https://blogger.googleusercontent.com/img/a/AVvXsEi0RNwo4uuNx4bcX3GVwz_00e11laD205bfuqrXuf7TnLwRzzGcmtbXiSUYokJ1-zAWvVcdN7SpRLkKz9em_-c8enpqd2COKwzNKLPLz9elinFyj9XIu1w7Wf78_9nz4VQ2CGZwGGKBCIv2qJltoT4NyzX6XjyBYmEA27x91PTu0XJVn0VZeMRclPEK=s320" width="240" /></a></div><br /><div class="separator" style="clear: both; text-align: center;"><br /></div><br /><span style="font-family: helvetica; font-size: large;"><br /></span><p></p><p><span style="font-family: helvetica; font-size: large;"><span style="background-color: white; color: #0f1111;">For smaller, non deep wounds, irritated skin I recently discovered this ointment, </span><span style="background-color: white; color: #0f1111;"><a href="https://www.amazon.com/Cetaphil-Gel-cream-Protectant-Dermatologist-Recommended/dp/B0897ZLQZM/ref=sr_1_5?crid=3T2UCL9BJY5DT&keywords=cetaphil+moisturizing+gel&qid=1643650893&rdc=1&sprefix=cetaphil+moisturizing+gel%2Caps%2C123&sr=8-5" target="_blank">Cetaphil Soothing Gel-Cream</a>. I found it does sting a little, but the pain doesn't last long. For me, it provided 24-moisture when used with bandages. About 12 hours without covering the area. It provided a cooling feeling as well. I would use this at any age. </span></span></p><p><span style="background-color: white; color: #0f1111;"></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEgjwnmSaOITZN10CJuPkgjqNFAIZGGLWMzE5_HWWgj8BFd7FGuY13B4D74Q8bCQzgjvnkt6fpzfennDiJQKL9JW7ZJwdAwaBsBnR84PdAPdwsG5wP7fkQ5HFqJS4LYzHqXPg2YLs2fNqavdwnL_GvG2Nsl2MWmMwsrCRx-qQOPI7URXlzgBA7rs-mzb=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="320" src="https://blogger.googleusercontent.com/img/a/AVvXsEgjwnmSaOITZN10CJuPkgjqNFAIZGGLWMzE5_HWWgj8BFd7FGuY13B4D74Q8bCQzgjvnkt6fpzfennDiJQKL9JW7ZJwdAwaBsBnR84PdAPdwsG5wP7fkQ5HFqJS4LYzHqXPg2YLs2fNqavdwnL_GvG2Nsl2MWmMwsrCRx-qQOPI7URXlzgBA7rs-mzb=s320" width="240" /></a></div><br /><span style="font-family: helvetica; font-size: large;"><br /></span><p></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">For deep wounds that need moisture to heal, I use <a href="https://www.amazon.com/Solosite-Hydrogel-Wound-Gel-PACK/dp/B00ATSSD4S/ref=sr_1_8_mod_primary_new?keywords=hydrogel&qid=1643651199&sbo=RZvfv%2F%2FHxDF%2BO5021pAnSA%3D%3D&sprefix=hydrogel%2Caps%2C125&sr=8-8" target="_blank">hydrogel,</a> any brand. It is long last and is safe to use on large areas or open or deep wounds.</span></span></p><p><span style="background-color: white; color: #0f1111;"></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEig6fT8hfGp8tYzaRy4ZQJ3dc3QcFUuE5PziuDndt-k4-DXJcCNVg6iHqvDxvsLXRoPYThoMhJ6gRboALAHP-H4jcnbJI40-NDlpGmAlYMMvzRoE8UPm8PE6gfFuAhjIbU3kPKQDdh2hH-lT6BYVGv49JZgeymsBxcqnXOVl04s1ZU7PXPD1NlPChkE=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="320" src="https://blogger.googleusercontent.com/img/a/AVvXsEig6fT8hfGp8tYzaRy4ZQJ3dc3QcFUuE5PziuDndt-k4-DXJcCNVg6iHqvDxvsLXRoPYThoMhJ6gRboALAHP-H4jcnbJI40-NDlpGmAlYMMvzRoE8UPm8PE6gfFuAhjIbU3kPKQDdh2hH-lT6BYVGv49JZgeymsBxcqnXOVl04s1ZU7PXPD1NlPChkE=s320" width="240" /></a></div><br /><span style="font-family: helvetica; font-size: large;">For the thick callouses we have on our feet (common in generalized severe EBS) we found that <a href="https://www.amazon.com/Kerasal-Intensive-Healing-Ointment-Cracked/dp/B08TQMNSJ6/ref=sr_1_2_sspa?crid=1IW9VPHTDI88I&keywords=kerasal&qid=1643653120&sprefix=kerasol%2Caps%2C136&sr=8-2-spons&psc=1&spLa=ZW5jcnlwdGVkUXVhbGlmaWVyPUEyNFlEWVhFWlFPNEU1JmVuY3J5cHRlZElkPUEwNDY3NTY0MTdNM1VNTEZPWktKTiZlbmNyeXB0ZWRBZElkPUEwNDkwMjAxMzUxT0JKN1Q2SEc3RyZ3aWRnZXROYW1lPXNwX2F0ZiZhY3Rpb249Y2xpY2tSZWRpcmVjdCZkb05vdExvZ0NsaWNrPXRydWU=" target="_blank">Kerasal</a> Foot ointment is helpful in reducing the roughness of the callouses. I wouldn't use anywhere near an open wound though</span><p></p><p><span style="background-color: white; color: #0f1111;"></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEgdgSExkXvKBO_r_mofxDT2tKpFyRCvf-hxKbNYbowYmIQQZIvEl67JITSnUtnUZJcsgg1YYjNIG9v-hnQ64yHcbm4CJzLePzXo12SDQcU8xn0mjvs-fcx78kGImdmvsDBegpeTQ1l7bM-93OuwTigJQ-9fsrcweD23-ESkXDFV-XJm014FOn1Nxi47=s4608" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="4608" data-original-width="3456" height="320" src="https://blogger.googleusercontent.com/img/a/AVvXsEgdgSExkXvKBO_r_mofxDT2tKpFyRCvf-hxKbNYbowYmIQQZIvEl67JITSnUtnUZJcsgg1YYjNIG9v-hnQ64yHcbm4CJzLePzXo12SDQcU8xn0mjvs-fcx78kGImdmvsDBegpeTQ1l7bM-93OuwTigJQ-9fsrcweD23-ESkXDFV-XJm014FOn1Nxi47=s320" width="240" /></a></div><br /><span style="font-family: helvetica; font-size: large;"><br /></span><p></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">Now the following is what I use on myself and wouldn't use it on an infant or small child that isn't able to communicate well.</span></span></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">I struggle with being colonized with bacteria and now mater how often I shower or what soap i use, it is always there. It doesn't cause issues unless there is an open wound. But sometimes it cause me to itch horribly for no reason. I experiments with many different variations. </span></span></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">With no open wound, dead or dry skin, I use just plain Vick's vapor rub. When i am dealing with excessive itchy, flaking, infected wounds or excessive bacteria on the skin, I made the following ointment:</span></span></p><p><span style="background-color: white; color: #0f1111;"></span></p><div class="separator" style="clear: both; text-align: center;"><a href="https://blogger.googleusercontent.com/img/a/AVvXsEjqL58_9RiwNXdehaGW8jTTz2sIdUZdwM9nIYn3qMqteFVHDAik5oCXS5C5lXz5lafD5is-FCFgTKbNsXiIaQzjTVx1ZeuhJrecO0vGqT-rBjpQsJBVMKyN20Nm8pfjfdsStyD0ohCeF_U9a036JJfBK1i3nzs4bCAAhS5eSt7s_S3q1ARsKcXv1XHA=s504" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="504" data-original-width="457" height="235" src="https://blogger.googleusercontent.com/img/a/AVvXsEjqL58_9RiwNXdehaGW8jTTz2sIdUZdwM9nIYn3qMqteFVHDAik5oCXS5C5lXz5lafD5is-FCFgTKbNsXiIaQzjTVx1ZeuhJrecO0vGqT-rBjpQsJBVMKyN20Nm8pfjfdsStyD0ohCeF_U9a036JJfBK1i3nzs4bCAAhS5eSt7s_S3q1ARsKcXv1XHA=w213-h235" width="213" /></a></div><br /><span style="font-family: helvetica; font-size: large;"><br /></span><p></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">4oz <a href="https://www.target.com/p/vicks-vaporub-cough-suppressant-ointment/-/A-14886279?preselect=13327570#lnk=sametab" target="_blank">vick's vapor rub</a></span></span></p><p><span style="font-family: helvetica; font-size: large;"><span style="background-color: white; color: #0f1111;">1/2 tube of <a href="https://www.target.com/p/polysporin-first-aid-antibiotic-ointment-0-5oz/-/A-13404340#lnk=sametab" target="_blank">polysporin</a> antibiotic ointment</span><span style="background-color: white; color: #0f1111;"> </span></span></p><p><span style="background-color: white; color: #0f1111;"><span style="font-family: helvetica; font-size: large;">1/2 tube of <a href="https://www.target.com/p/anti-itch-1-hydrocortisone-maximum-strength-intensive-healing-cream-1oz-up-38-up-8482/-/A-13976189#lnk=sametab">hydrocortisone</a> cream</span></span></p><p><span style="font-family: helvetica; font-size: large;"><span style="background-color: white; color: #0f1111;">1/2 tube of </span><span style="background-color: white; color: #333333;"><a href="https://www.target.com/p/hemorrhoid-treatment-cream-1-8oz-up-38-up-8482/-/A-81404365#lnk=sametab" target="_blank">Hemorrhoid Treatment Cream</a> (for pain relief)</span></span></p><p><span style="font-family: helvetica; font-size: large;">I mix it all together. I use it mostly on my feet and ankles. It helps with itching and pain. I use under bandages and change daily, and clean the areas very well daily in-between bandage changes. I have tried it on large open wounds and not only does it sting, I saw no benefit form it in regards to healing. Sometimes I'll change out the antibiotic ointment for something stronger, depending on what bacteria I am having issues with. This is one I would NOT use on infants or children. I am willing to risk any long term side effects I may suffer on myself, but not my kids. </span></p><p><span style="font-family: helvetica; font-size: large;"> </span></p><p><span style="font-family: helvetica; font-size: large;">When it comes to wound ointments, it really comes down to personal preference, comfort and what works for that particular person. But it always good to know what is out there and what works for others.<br /></span></p><p><span style="font-size: large;"><br /></span></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-37088529591306863302021-07-28T07:54:00.003-04:002021-08-09T11:55:17.077-04:00The range in severity in EB simplex. <p style="text-align: center;"><i><span style="font-family: Karla; font-size: medium;"><b><span style="background-color: white;">This post is dedicated to <a href="https://memorials.snowfuneralhome.net/inez-elizabeth-raquel-ontiveros-jaime/4675139/index.php?_ga=2.190322411.1134886817.1627346538-754520536.1626448295" target="_blank">Inez Elizabeth Raquel Ontiveros Jaime</a> who lost her battle to EB Simplex while I was composing this post. </span></b></span></i></p><p style="text-align: center;"><i><span style="font-family: Quicksand; font-size: medium;"><b><span style="background-color: white; color: #626262;"><br /></span></b></span></i></p><p style="text-align: center;"><i><span style="font-family: Quicksand; font-size: medium;"><b></b></span></i></p><div class="separator" style="clear: both; text-align: center;"><i><span style="font-family: Quicksand; font-size: medium;"><b><a href="https://1.bp.blogspot.com/-Iz_Iu-A77JY/YRFOxgt4PfI/AAAAAAAAnQI/maHINaSaL1YSSx0ECKnPUgbr3JoT6WEWQCLcBGAsYHQ/s715/received_216558033651741.jpeg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="715" data-original-width="585" height="200" src="https://1.bp.blogspot.com/-Iz_Iu-A77JY/YRFOxgt4PfI/AAAAAAAAnQI/maHINaSaL1YSSx0ECKnPUgbr3JoT6WEWQCLcBGAsYHQ/w164-h200/received_216558033651741.jpeg" width="164" /></a></b></span></i></div><i><span style="font-family: Quicksand; font-size: medium;"><b><br /><span style="background-color: white; color: #626262;"><br /></span></b></span></i><p></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span>When people hear the term EB simplex, they likely of someone with mild blisters on their hands and feet. Nothing serious, nothing compared to the other more severe forms of EB. </span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span>And while simplex, is milder than some of the other forms of EB, the range in severity can vary GREATLY from very mild to very severe. The purpose of this post is to show the serenity within the umbrella of EB simplex. I am NOT comparing it to other forms of EB. </span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><b>These are dominant forms of EB Simplex </b><br /></span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span> </span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><b><span>EB Simplex- Localized (formally known as <span><span class="teaser">Weber-Cockayne)</span></span></span></b></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span>Per the Genetic and Rare Disease Website: Onset is usually in late infancy or early childhood. The usual
distribution of blisters in these patients is on the palms and soles,
although other skin surfaces may also blister if subjected to
significant trauma. Milia and scarring are rare in localized EBS, and
dystrophic nails are uncommon. Focal keratoderma (thickening) of the palms and soles
may occur by adulthood in some patients. The only common extracutaneous
finding in localized EBS, i.e. localized oral erosions or blisters,
tends to be asymptomatic, occurs in about one third of patients, and is
usually seen only during infancy. EBS-LOC is autosomal dominant and sporadic cases are frequent. Although the disease can be disabling, life-expectancy is normal.</span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><a href="https://1.bp.blogspot.com/-u2wswUfPpXE/YNppx4YXqvI/AAAAAAAAmJc/1D9Ivw1V7OU48hr5CPHMLm48jQvUw3EbACLcBGAsYHQ/s960/ebs_wc1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="960" data-original-width="720" height="200" src="https://1.bp.blogspot.com/-u2wswUfPpXE/YNppx4YXqvI/AAAAAAAAmJc/1D9Ivw1V7OU48hr5CPHMLm48jQvUw3EbACLcBGAsYHQ/w150-h200/ebs_wc1.jpg" width="150" /></a></span></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><a href="https://1.bp.blogspot.com/-5j4-eddQ0T4/YNppyuoRFqI/AAAAAAAAmJo/rwdWebAGGHYVgme4-vM9u4dIZiqco6hcQCLcBGAsYHQ/s960/ebs_wc4.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="960" data-original-width="720" height="200" src="https://1.bp.blogspot.com/-5j4-eddQ0T4/YNppyuoRFqI/AAAAAAAAmJo/rwdWebAGGHYVgme4-vM9u4dIZiqco6hcQCLcBGAsYHQ/w150-h200/ebs_wc4.jpg" width="150" /></a></span></span></span></div></div><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span><table align="center" cellpadding="0" cellspacing="0" class="tr-caption-container" style="margin-left: auto; margin-right: auto;"><tbody><tr><td style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><a href="https://1.bp.blogspot.com/-kCMAUnIKvhY/YNppx-tI2uI/AAAAAAAAmJg/seLUsN-lGtsIub7c9cusY86MrLXP8M7sQCLcBGAsYHQ/s1440/ebs_ewc2.jpg" style="margin-left: auto; margin-right: auto;"><img border="0" data-original-height="1080" data-original-width="1440" height="150" src="https://1.bp.blogspot.com/-kCMAUnIKvhY/YNppx-tI2uI/AAAAAAAAmJg/seLUsN-lGtsIub7c9cusY86MrLXP8M7sQCLcBGAsYHQ/w200-h150/ebs_ewc2.jpg" width="200" /></a></span></span></span></td></tr><tr><td class="tr-caption" style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span></td></tr></tbody></table><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><a href="https://1.bp.blogspot.com/-OncETgvGsAE/YNppyDzIJcI/AAAAAAAAmJk/3AuKOVsKQ3IxCBbw6Iq4x-GRj3JjcURfwCLcBGAsYHQ/s2015/ebs.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="2015" data-original-width="1504" height="200" src="https://1.bp.blogspot.com/-OncETgvGsAE/YNppyDzIJcI/AAAAAAAAmJk/3AuKOVsKQ3IxCBbw6Iq4x-GRj3JjcURfwCLcBGAsYHQ/w149-h200/ebs.jpg" width="149" /></a><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-C_2moXM2LyA/YNpwODdB92I/AAAAAAAAmJ8/u3-GlIrbR9w4vD2-hBsUhQ27HI3Q-n1QACLcBGAsYHQ/s455/wbs_wc3.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="455" data-original-width="342" height="200" src="https://1.bp.blogspot.com/-C_2moXM2LyA/YNpwODdB92I/AAAAAAAAmJ8/u3-GlIrbR9w4vD2-hBsUhQ27HI3Q-n1QACLcBGAsYHQ/w151-h200/wbs_wc3.jpg" width="151" /></a></div></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span>Those are probably what people picture in their head when they hear the phrase "EB simplex". Below is what EB simplex can also look like....</span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span><br /></span></span></span></div><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span>*************************************************************<br /></span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><b><span>EBS- generalized intermediate (formerly know as Koebner) </span></b></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span>Per the Genetic and Rare Disease Website: Epidermolysis bullosa simplex, generalized intermediate is associated with widespread blisters that appear at birth or in early infancy.Blisters occur in areas where skin rubs together, or after minor
injuries. Other features of EBS include nail involvement, thickening of
the skin on the palms of the hands and soles of the feet, and small
bump-like cysts known as milia The symptoms of this condition can be very different from person to
person, ranging from mild blistering of the hands and feet to blistering
that occurs all over the body that can be fatal. This form can either be dominant or recessive in inheritance.</span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span>Epidermolysis bullosa simplex (EBS) generalized intermediate that is associated with genetic changes in either the <i>KRT5</i> or <i>KRT14</i> genes is inherited in an autosomal dominant pattern. Epidermolysis bullosa simplex (EBS) generalized intermediate that is associated with genetic changes in either the <i>EXPH5</i> or <i>TGM5</i> genes is inherited in an autosomal recessive pattern</span></span></span></p><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-s0QwmOX7qD4/YNpwhu7p1AI/AAAAAAAAmKY/OvYYFAlU5O46GIwAee2J088Pmk4Twm6qwCLcBGAsYHQ/s2048/EBS_k1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="2048" data-original-width="1536" height="200" src="https://1.bp.blogspot.com/-s0QwmOX7qD4/YNpwhu7p1AI/AAAAAAAAmKY/OvYYFAlU5O46GIwAee2J088Pmk4Twm6qwCLcBGAsYHQ/w150-h200/EBS_k1.jpg" width="150" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-AaNvZNgIQuA/YNpwhs9b7BI/AAAAAAAAmKc/JIfF70uMdJAXID7U4VCtC6HeOd7iIOxegCLcBGAsYHQ/s2048/ebs_k2.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1536" data-original-width="2048" height="150" src="https://1.bp.blogspot.com/-AaNvZNgIQuA/YNpwhs9b7BI/AAAAAAAAmKc/JIfF70uMdJAXID7U4VCtC6HeOd7iIOxegCLcBGAsYHQ/w200-h150/ebs_k2.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-6GwR5sy9WWI/YNpwhtK8xhI/AAAAAAAAmKU/S9GV4hm0muI1R09bvL-VhwLHMzq82WOggCLcBGAsYHQ/s2048/ebs_k3.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1536" data-original-width="2048" height="150" src="https://1.bp.blogspot.com/-6GwR5sy9WWI/YNpwhtK8xhI/AAAAAAAAmKU/S9GV4hm0muI1R09bvL-VhwLHMzq82WOggCLcBGAsYHQ/w200-h150/ebs_k3.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-XI3ftBiFr9k/YNpwiDr8hKI/AAAAAAAAmKg/FPivhOT7Uis3gwuEnW36gn5OJT6EeLxwQCLcBGAsYHQ/s1104/ebs_k4.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1104" data-original-width="828" height="200" src="https://1.bp.blogspot.com/-XI3ftBiFr9k/YNpwiDr8hKI/AAAAAAAAmKg/FPivhOT7Uis3gwuEnW36gn5OJT6EeLxwQCLcBGAsYHQ/w150-h200/ebs_k4.jpg" width="150" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-hYHcaAqPko0/YNpwiczsoXI/AAAAAAAAmKk/7R4JcB6VQbQIaIQGeheh4dr_6fF7Hjj_wCLcBGAsYHQ/s1104/ebs_k5.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1104" data-original-width="828" height="200" src="https://1.bp.blogspot.com/-hYHcaAqPko0/YNpwiczsoXI/AAAAAAAAmKk/7R4JcB6VQbQIaIQGeheh4dr_6fF7Hjj_wCLcBGAsYHQ/w150-h200/ebs_k5.jpg" width="150" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /> <br /></span></span><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span> **********************************************************</span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><b><span>EBS- generalized severe (formerly known as dowling meara) </span></b></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span><span>Per the Genetic and Rare Disease Website: </span>Onset is usually at birth with large, frequently hemorrhagic blisters.
After the neonatal period, the lesions take the typical herpetiform (or
herpes-like) clustering with central healing pattern. Blister formation
gradually reduces starting from late childhood. By childhood, most
patients begin to develop confluent thickening and hyperkeratosis
(keratoderma) of the palms and soles which may partially resolve in some
patients during mid- to late-adulthood. Along with blisters, skin
findings commonly include mild atrophic scarring and post-inflammatory
pigmentation, nail shedding and nail dystrophy, as well as occasional
milia formation. Lesions may improve in some patients in case of fever,
unlike other forms of EB in which warmer weather exacerbates disease
activity. The reason for this is unknown. Extracutaneous complications
can occur including oral cavity blistering, constipation and, rarely,
tracheolaryngeal compromise. </span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span>Transmission is autosomal dominant and sporadic cases are frequent.</span></span></span></p><div><span style="font-family: Roboto;"><span style="font-size: medium;"><span>EBS- generalized severe is frequently associated with marked morbidity in infancy and early
childhood and, in rare cases, may result in death during early infancy.
Patients also have a markedly increased risk of basal cell carcinoma by mid-adulthood (cumulative risk of 44% by age 55).</span></span></span></div><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span> </span></span></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-hxcyehstVD8/YNpwx7uNENI/AAAAAAAAmK4/9ONBXi7adxE0f99cnvB_7J-5lTyjKogfQCLcBGAsYHQ/s173/easton_2.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="167" data-original-width="173" src="https://1.bp.blogspot.com/-hxcyehstVD8/YNpwx7uNENI/AAAAAAAAmK4/9ONBXi7adxE0f99cnvB_7J-5lTyjKogfQCLcBGAsYHQ/s0/easton_2.jpg" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-9KOldcVmgS8/YNpwyJ3pJzI/AAAAAAAAmLA/wzqQThflB7IvmcbTb8RbnumOEi4Bj6K6QCLcBGAsYHQ/s670/easton_3.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="538" data-original-width="670" height="161" src="https://1.bp.blogspot.com/-9KOldcVmgS8/YNpwyJ3pJzI/AAAAAAAAmLA/wzqQThflB7IvmcbTb8RbnumOEi4Bj6K6QCLcBGAsYHQ/w200-h161/easton_3.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-3xBoNtamXtM/YNpwyCBz8OI/AAAAAAAAmK8/IIl6zAk3o-E_BT0xHeWyGcQAmf-p0UT-gCLcBGAsYHQ/s642/easton_4.1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="642" data-original-width="580" height="200" src="https://1.bp.blogspot.com/-3xBoNtamXtM/YNpwyCBz8OI/AAAAAAAAmK8/IIl6zAk3o-E_BT0xHeWyGcQAmf-p0UT-gCLcBGAsYHQ/w181-h200/easton_4.1.jpg" width="181" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-y6CKFGtfomI/YNpwyyTYg5I/AAAAAAAAmLE/3xkllnxgYbkzwJMHrO-8rWl7cEQip45wQCLcBGAsYHQ/s311/easton_4.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="311" data-original-width="282" height="200" src="https://1.bp.blogspot.com/-y6CKFGtfomI/YNpwyyTYg5I/AAAAAAAAmLE/3xkllnxgYbkzwJMHrO-8rWl7cEQip45wQCLcBGAsYHQ/w181-h200/easton_4.jpg" width="181" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-sM_6Pjgof2o/YNpwzJX0UII/AAAAAAAAmLI/t8Yq-mlG8TINCo7DaNIMLAYf5mCZ38QvgCLcBGAsYHQ/s426/easton_5.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="320" data-original-width="426" height="150" src="https://1.bp.blogspot.com/-sM_6Pjgof2o/YNpwzJX0UII/AAAAAAAAmLI/t8Yq-mlG8TINCo7DaNIMLAYf5mCZ38QvgCLcBGAsYHQ/w200-h150/easton_5.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-atX2KJLAw74/YNpwzl_IUSI/AAAAAAAAmLM/unBfiiL5pdA2HgH5QfmUkpJ_a2vnf_tmACLcBGAsYHQ/s960/easton_6.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="720" data-original-width="960" height="150" src="https://1.bp.blogspot.com/-atX2KJLAw74/YNpwzl_IUSI/AAAAAAAAmLM/unBfiiL5pdA2HgH5QfmUkpJ_a2vnf_tmACLcBGAsYHQ/w200-h150/easton_6.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-wGeSKuSDb2Q/YNpw0A0igtI/AAAAAAAAmLQ/aTfjZVyAwC485suJz6K32q9oWf1BPUBqgCLcBGAsYHQ/s160/easton_7.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="109" data-original-width="160" height="136" src="https://1.bp.blogspot.com/-wGeSKuSDb2Q/YNpw0A0igtI/AAAAAAAAmLQ/aTfjZVyAwC485suJz6K32q9oWf1BPUBqgCLcBGAsYHQ/w200-h136/easton_7.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-M4Ayy8-_ALA/YNpw1F0FylI/AAAAAAAAmLY/Y6kV0_2nY9EK2B8or4DhaeghOJlXIxKpwCLcBGAsYHQ/s720/joey_foot2.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="540" data-original-width="720" height="150" src="https://1.bp.blogspot.com/-M4Ayy8-_ALA/YNpw1F0FylI/AAAAAAAAmLY/Y6kV0_2nY9EK2B8or4DhaeghOJlXIxKpwCLcBGAsYHQ/w200-h150/joey_foot2.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-wYERGMt2o8I/YNpw1vEmuuI/AAAAAAAAmLc/B-vebneZ3Iw-nZcfNkfvAqHYKezrgJS9wCLcBGAsYHQ/s640/joey_foot3.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="480" data-original-width="640" height="150" src="https://1.bp.blogspot.com/-wYERGMt2o8I/YNpw1vEmuuI/AAAAAAAAmLc/B-vebneZ3Iw-nZcfNkfvAqHYKezrgJS9wCLcBGAsYHQ/w200-h150/joey_foot3.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-YpzmdySNWeg/YNpw0rbgOTI/AAAAAAAAmLU/HdJNIV3iCmQnHrV6PK_1GCenqiiLc6LEgCLcBGAsYHQ/s720/joey_foot.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="540" data-original-width="720" height="150" src="https://1.bp.blogspot.com/-YpzmdySNWeg/YNpw0rbgOTI/AAAAAAAAmLU/HdJNIV3iCmQnHrV6PK_1GCenqiiLc6LEgCLcBGAsYHQ/w200-h150/joey_foot.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-8AUxrd-t8UA/YNpw2KKEKyI/AAAAAAAAmLg/bzaaQwoFIJ8D9PN17uotscrXPlldalA8gCLcBGAsYHQ/s720/joey_hand.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="540" data-original-width="720" height="150" src="https://1.bp.blogspot.com/-8AUxrd-t8UA/YNpw2KKEKyI/AAAAAAAAmLg/bzaaQwoFIJ8D9PN17uotscrXPlldalA8gCLcBGAsYHQ/w200-h150/joey_hand.jpg" width="200" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-Yr6E7jowmw4/YNpw3Fa839I/AAAAAAAAmLo/f1QH01l5n6kFoUnLC73fcgu8n9sa_xRHACLcBGAsYHQ/s512/joey_leg.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="385" data-original-width="512" height="151" src="https://1.bp.blogspot.com/-Yr6E7jowmw4/YNpw3Fa839I/AAAAAAAAmLo/f1QH01l5n6kFoUnLC73fcgu8n9sa_xRHACLcBGAsYHQ/w200-h151/joey_leg.jpg" width="200" /></a></span></span></div><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-cTW9smdloqA/YPAj9fDiqZI/AAAAAAAAmoY/9jPL4mMiWiY_mtcMh731QYCXw1NGRyyFACLcBGAsYHQ/s2048/026.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="2048" data-original-width="1122" height="264" src="https://1.bp.blogspot.com/-cTW9smdloqA/YPAj9fDiqZI/AAAAAAAAmoY/9jPL4mMiWiY_mtcMh731QYCXw1NGRyyFACLcBGAsYHQ/w144-h264/026.jpg" width="144" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-T1zvSmx0mmE/YPAkB57ZN8I/AAAAAAAAmoc/aJ2ehTWlBZExEouYnIntdVf0BiCXVJ94wCLcBGAsYHQ/s2048/043.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1536" data-original-width="2048" height="185" src="https://1.bp.blogspot.com/-T1zvSmx0mmE/YPAkB57ZN8I/AAAAAAAAmoc/aJ2ehTWlBZExEouYnIntdVf0BiCXVJ94wCLcBGAsYHQ/w246-h185/043.jpg" width="246" /></a></span></span></div><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;">******************************************<br /></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"> </span></span></p><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"> <b>EBS-KLHL24 subtype</b><br /></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;">EBS KLHL24 have a characteristic clinical phenotype, showing skin
defects and blistering at birth and unusual stellate scarring, skin
fragility, and whorled or macular hyperpigmentation or hypopigmentation
in childhood. Although skin fragility improves by adulthood, nail dystrophy, anetoderma, and hair loss may occur. Patients with this type are also at high risk for cardiomyopathy </span></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-5zqXlBtPwOs/YPCQ62JkQ-I/AAAAAAAAmow/o68dc7M3S_8AzZHSG-c4e50LaajWaboFgCLcBGAsYHQ/s635/KLHL24.2.1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="635" data-original-width="273" height="320" src="https://1.bp.blogspot.com/-5zqXlBtPwOs/YPCQ62JkQ-I/AAAAAAAAmow/o68dc7M3S_8AzZHSG-c4e50LaajWaboFgCLcBGAsYHQ/s320/KLHL24.2.1.jpg" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><p><span style="font-family: Roboto;"><span style="font-size: medium;"><br /> <br /></span></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-ft-vp_AvgVI/YPCRETlnWhI/AAAAAAAAmo0/IoNokT6IUT8pJnA2IlRL7HuYq0-aGfr0gCLcBGAsYHQ/s652/KLHL24.1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="652" data-original-width="488" height="275" src="https://1.bp.blogspot.com/-ft-vp_AvgVI/YPCRETlnWhI/AAAAAAAAmo0/IoNokT6IUT8pJnA2IlRL7HuYq0-aGfr0gCLcBGAsYHQ/w206-h275/KLHL24.1.jpg" width="206" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-PROnFrPcPR4/YPCREaJlBvI/AAAAAAAAmo4/tpEzF94_4CQl-rhvuUUfiknaSJpmoWN0QCLcBGAsYHQ/s653/KLHL24.3.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="653" data-original-width="487" height="270" src="https://1.bp.blogspot.com/-PROnFrPcPR4/YPCREaJlBvI/AAAAAAAAmo4/tpEzF94_4CQl-rhvuUUfiknaSJpmoWN0QCLcBGAsYHQ/w202-h270/KLHL24.3.jpg" width="202" /></a></span></span></div><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"> ********************************************</span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"> </span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><b>Now onto the recessive forms of EB: </b></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><b> </b></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;">Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD)</span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;">Onset of blistering is usually as early as birth, whereas muscular
dystrophy manifests between infancy and adulthood. Blisters are often
hemorrhagic and heal with mild atrophic scarring and rare milia
formation. Associated findings comprise markedly dystrophic nails, and
focal keratoderma of the palms and soles. Extracutaneous involvement is
usually present, including enamel hypoplasia with premature tooth decay,
blistering in the oral cavity, pharynx and, rarely, larynx and trachea
with inspiratory stridor and breathing difficulties requiring
tracheotomy. Slowly progressive weakness of the head and limb muscles
appears between the first year and the fourth decade of life and may
confine the patient to a wheelchair. Additional neurological symptoms (ptosis, oculobulbar muscle weakness and fatigability) indicative of a myasthenic syndrome have been described in some patients. EBS-MD is caused by mutations in the <i>PLEC</i> gene (8q24) encoding plectin. Plectin deficiency can be demonstrated in skin and muscle by analysis with specific antibodies.</span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"> </span></span></p><p><span style="font-family: Roboto;"></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-WWcUi_98EtE/YPCT_9ufxsI/AAAAAAAAmpc/9sks4t7ftYQHEc8BmWGgjDrYx3b5zyjPQCLcBGAsYHQ/s1000/ebs-md.1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1000" data-original-width="750" height="229" src="https://1.bp.blogspot.com/-WWcUi_98EtE/YPCT_9ufxsI/AAAAAAAAmpc/9sks4t7ftYQHEc8BmWGgjDrYx3b5zyjPQCLcBGAsYHQ/w172-h229/ebs-md.1.jpg" width="172" /></a></span></span></div><p><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-kC6EbtY8Arw/YPCUAOdCnTI/AAAAAAAAmpk/umIm-38T-lEwpPT9mwiSjtG1YhHW2LZ2wCLcBGAsYHQ/s2048/ebs-md.2.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="2048" data-original-width="1536" height="225" src="https://1.bp.blogspot.com/-kC6EbtY8Arw/YPCUAOdCnTI/AAAAAAAAmpk/umIm-38T-lEwpPT9mwiSjtG1YhHW2LZ2wCLcBGAsYHQ/w169-h225/ebs-md.2.jpg" width="169" /></a></span></div><span style="font-size: medium;"><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-w7ZVOJDnz_E/YPCT_12DxGI/AAAAAAAAmpg/7JqeuzVTMjsxdJlhOnu7Lnp2_3eCnRRIwCLcBGAsYHQ/s750/ebs-md.3.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="563" data-original-width="750" height="187" src="https://1.bp.blogspot.com/-w7ZVOJDnz_E/YPCT_12DxGI/AAAAAAAAmpg/7JqeuzVTMjsxdJlhOnu7Lnp2_3eCnRRIwCLcBGAsYHQ/w249-h187/ebs-md.3.jpg" width="249" /></a></div><br /><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-YBrkg4Mqh_c/YPCUAY_jZBI/AAAAAAAAmpo/kRxSSAFuJSsZ2rB9Bi2ZKDoPlKDjNmY0gCLcBGAsYHQ/s1000/ebs-md.4.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1000" data-original-width="750" height="249" src="https://1.bp.blogspot.com/-YBrkg4Mqh_c/YPCUAY_jZBI/AAAAAAAAmpo/kRxSSAFuJSsZ2rB9Bi2ZKDoPlKDjNmY0gCLcBGAsYHQ/w187-h249/ebs-md.4.jpg" width="187" /></a></div><br /></span><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-9tcydVFIsJE/YQFEpoPnXqI/AAAAAAAAm-Y/nDYy3ZfhMHgzYPWQirD8qf-iNYsYRws-QCLcBGAsYHQ/s1000/EBS-md5.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="1000" data-original-width="750" height="277" src="https://1.bp.blogspot.com/-9tcydVFIsJE/YQFEpoPnXqI/AAAAAAAAm-Y/nDYy3ZfhMHgzYPWQirD8qf-iNYsYRws-QCLcBGAsYHQ/w208-h277/EBS-md5.jpg" width="208" /></a></div><br /><p></p><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><span style="-webkit-text-stroke-width: 0px; background-color: white; color: #777777; display: inline; float: none; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; letter-spacing: normal; text-align: left; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"> *******************************************</span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span style="-webkit-text-stroke-width: 0px; background-color: white; color: #777777; display: inline; float: none; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; letter-spacing: normal; text-align: left; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"> </span></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;"><span style="-webkit-text-stroke-width: 0px; background-color: white; color: #777777; display: inline; float: none; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; letter-spacing: normal; text-align: left; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;">Autosomal </span>Recessive EBS- K14 mutation: <br /></span></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><span style="font-size: medium;">Skin blistering starts at birth and is generalized and severe in most cases. No improvement of cutaneous fragility is expected with age. Healing of lesions leads to post-inflammatory hyperpigmentation. </span></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><span style="font-size: medium;"> </span></span></p><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-dkKyUfHmc6Q/YPCWFBfSBYI/AAAAAAAAmp4/i13XWO8gMH0AMQpTFtwVGdIRgE14ltRIQCLcBGAsYHQ/s2048/ebs-r.1.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="2048" data-original-width="1535" height="320" src="https://1.bp.blogspot.com/-dkKyUfHmc6Q/YPCWFBfSBYI/AAAAAAAAmp4/i13XWO8gMH0AMQpTFtwVGdIRgE14ltRIQCLcBGAsYHQ/s320/ebs-r.1.jpg" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><div class="separator" style="clear: both; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;"><a href="https://1.bp.blogspot.com/-_DwiSU5svnU/YPCWFB4lJUI/AAAAAAAAmp8/QsC4n9hKkzwu5NYrVa6CWIks8FBzjbBqwCLcBGAsYHQ/s2048/ebs-r.2.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="2048" data-original-width="1536" height="320" src="https://1.bp.blogspot.com/-_DwiSU5svnU/YPCWFB4lJUI/AAAAAAAAmp8/QsC4n9hKkzwu5NYrVa6CWIks8FBzjbBqwCLcBGAsYHQ/s320/ebs-r.2.jpg" /></a></span></span></div><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><span style="font-size: medium;"><br /></span></span></p></li></ul><p style="text-align: center;"><span style="font-family: Roboto;"><span style="font-size: medium;">Other Rare Types of EBS (info obtained from www.debra.org)<br /></span></span></p><p><span style="font-family: Roboto;"><span style="font-size: medium;">EBS with mottled pigmentation: </span></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Skin blistering starts at birth and is generalized, of intermediate severity. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Mottled or reticulate pigmentation develops gradually </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Focal keratoses of the palms and soles, and dystrophic, thickened nails occur over time. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: left; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal dominant inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">The keratin 5 monoallelic pathogenic variant c.74C>T, p.P25L typically causes this phenotype,13 but cases with other variants in KRT5, KRT14 or EXPH5 have been reported. </span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><b> </b></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><b> **********************************</b></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><b>EBS with migratory circinate eythema</b></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: left; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Multiple vesicles are present from birth onwards and acquire over time a typical circinate migratory pattern on an erythematous background; post-inflammatory hyperpigmentation develops gradually and may have a mottled pattern. </span></p></li></ul></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Nails may be dystrophic. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal dominant inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Monoallelic pathogenic variants in KRT5 which affect the variable 2 domain and result in frameshift and elongated keratin 5 polypeptide cause this phenotype. </span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><b>EBS, localized or intermediate with BP230 deficiency</b></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Skin blistering starts at birth or in childhood and is mostly localized to acral extremities. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Plantar keratoderma. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Nail dystrophy. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="4" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal recessive inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Biallelic loss-of-function pathogenic variants in DST lead to absence of BP230 and cause this subtype. </span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><b>EBS, localized or intermediate with exophilin 5 deficiency</b></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Generalized skin blistering starts at birth or in infancy. Blistering tendency may diminish with age, while crusts and scabs reflect the fragility of the skin. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Mild mottled pigmentary changes may develop. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal recessive inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Biallelic loss-of-function pathogenic variants in EXPH5 lead to absence of exophilin 5 and cause this subtype. <br /></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">EBS, intermediate with PLEC variants</span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Skin blistering starts at birth, is mainly acral but may be widespread. The autosomal dominant subtype is characterized by a mild course, mainly acral erosions and postlesional violaceous and hypopigmented macules. Only three cases with the autosomal recessive subtype have been published yet, all of intermediate severity. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Plantar keratoderma. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Dystrophic thickened nails, sometimes onychogryphosis. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="4" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">No muscular dystrophy. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal dominant or recessive inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">The monoallelic PLEC pathogenic variant, c.5998C>T, p.R2000W causes the autosomal dominant subtype previously known as EBS Ogna. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Biallelic pathogenic variants in the exon 1a of the PLEC1a isoform (expressed in skin, but not in muscles), in particular, c.46C>T, p.R16*, cause the autosomal recessive subtype. </span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">EBS, severe with pyloric atresia<br /></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"> </span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Widespread full-thickness congenital absence of skin. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Pyloric atresia. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Involvement of the oral mucosa. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="4" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Anemia and growth retardation. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="5" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Neonatal lethal course. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal recessive inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Biallelic loss-of-function pathogenic variants in PLEC cause this phenotype. <br /></span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><span style="font-family: Roboto;"><br /></span></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">EBS, localized with nephropathy w/ CD151 deficiency</span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;"><br /></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Only a few individuals with this subtype have been reported so far in the literature. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Skin blistering starts at birth and is widespread primarily in the pretibial area but also scattered on other parts of the body, particularly those exposed to trauma. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Facial freckling, poikiloderma and atrophy of the skin, and acrogeria of the backs of the hands on the sun-exposed areas reported in one case. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="4" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Erosions of the oral mucous membranes. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="5" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Nail dystrophy. </span></p></li></ul></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Early-onset alopecia. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Nasolacrimal duct stenosis. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="3" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Oesophageal webbing and strictures. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="4" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Nephropathy manifesting with proteinuria. The scarcity of reported cases precludes firm screening recommendations, but annual urinalysis and urea and electrolytes should probably be undertaken following diagnosis. </span></p></li></ul><p style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; line-height: 24px; margin: 0px 0px 20px; text-align: start; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><span style="font-family: Roboto;"><b style="box-sizing: border-box; font-weight: 700;">Genetics</b></span></p><ul role="list" style="-webkit-text-stroke-width: 0px; background-color: white; box-sizing: border-box; color: #777777; font-size: 16px; font-style: normal; font-variant-caps: normal; font-variant-ligatures: normal; font-weight: 400; letter-spacing: normal; margin-bottom: 2em; margin-left: 0px; margin-top: 1em; padding-left: 25px; text-align: left; text-decoration-color: initial; text-decoration-style: initial; text-decoration-thickness: initial; text-indent: 0px; text-transform: none; white-space: normal; word-spacing: 0px;"><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="1" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Autosomal recessive inheritance. </span></p></li><li aria-setsize="-1" data-aria-level="4" data-aria-posinset="2" data-font="Symbol" data-leveltext="" data-listid="2" role="listitem" style="box-sizing: border-box; line-height: 19.2px; margin-bottom: 0.5em;"><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto;">Biallelic loss-of-function pathogenic variants in CD151, coding for the CD151 antigen cause this EBS subtype. </span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px;"><span style="font-family: Roboto; font-size: x-large;"><br /></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px; text-align: center;"><span style="font-size: x-large;"><b><span style="font-family: Roboto;"><br /></span></b></span></p><p style="box-sizing: border-box; color: #777777; line-height: 24px; margin: 0px; text-align: center;"><span style="font-family: Roboto;"><span style="font-size: x-large;"><b>For those that made it this far, THANK YOU for reading this post!!! My purpose is to educate and inform. </b></span><br /></span></p></li></ul>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com1tag:blogger.com,1999:blog-7961655801018531468.post-16393458037486772862021-01-22T23:38:00.004-05:002021-01-22T23:50:25.065-05:00Junctional EB in Depth <p><b><span style="font-family: Poppins;">Junctional Epidermolysis Bullosa is an autosomal recessive disorder. The most prominent characteristic is the lack of tooth enamel which effects all sub-types of Junctional EB.</span></b></p><p><span style="font-family: Poppins;"><br /></span></p><p><span style="font-family: Poppins;">There are two main sub-types of Junctional EB (JEB)- generalized and localized.</span></p><p><span style="font-family: Poppins;"> </span></p><p><span style="font-family: Poppins;"><u><b>Generalized</b></u><br /></span></p><p><span style="font-family: Poppins;"><b>Generalized Severe JEB (JEB-sev gen)</b>- the protein affected is the Laminin 332 protein</span></p><p><span style="font-family: Poppins;"><b>Common Features: </b>the appearance of symmetrical granulation tissue around the mouth and nose is a significant, as is non healing wounds on the back of the head, the bottom and upper part of the back. The eyes, upper respiratory tract and genitals are also affected. Anemia, malnutrition, dehydration and airway obstruction are common and the leading cause of death for this subtype. <b><br /></b></span></p><p><br /></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b>〰</b></span></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"></span></p><p><span style="font-family: Poppins;"><b>Generalized Intermediate JEB (JEB- gen intermed)</b>- the proteins affected are Laminin 332 and Collagen 17</span></p><p><span style="font-family: Poppins;"><b>Common Features: </b>Blistering or massive skin loss present at birth; generalized blistering, dysphoric or absent nails, and post-inflammatory hypo-pigmentation is common throughout life. Alopecia is also common. There is a lower risk of death due to anemia, malnutrition and airway occlusion, but it can occur in some patients. There have been some cases of squamous cell carcinoma reported in patients with this type. <b><br /></b></span></p><p style="text-align: center;"><span style="font-family: Poppins;"> </span><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b>〰</b></span></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span></p><p><span style="font-family: Poppins;"><b>Junctional EB with pyloric atresia (JEB-PA)</b>- the protein affected is alpha6Beta4 Integrin </span></p><p><span style="font-family: Poppins;"><b>Common Features: </b>generalized blistering present at birth along with pyloric atresia. </span><span style="font-family: Poppins;"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0040">Congenital abnormalities of the genitourinary tract may also be present and severity can range from mild to fatal. </span></span></span></span></span></p><p><br /></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b> </b></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b>〰</b></span></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b> </b></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b> </b></span></span></p><p><span style="font-family: Poppins;"><u><b>Localized</b></u></span></p><p><span style="font-family: Poppins;"><b>Localized JEB( JEB-loc)-</b> the proteins affected are Laminin 332, Collagen 17 and Beta4 Integrin</span></p><p><span style="font-family: Poppins;"><b>Common Features: </b>blisters usually confined to the hands, feet and lower leg; nails are usually absent or dystrophic and dental abnormalities are common<b><br /></b></span></p><p style="text-align: center;"><span style="font-family: Poppins;"> </span><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b>〰</b></span></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span></p><p><span style="font-family: Poppins;"><b>JEB Inversa (JEB-inv)</b>- the protein affected is Laminin 332</span></p><p><span style="font-family: Poppins;"><b>Common Features: </b>this rare type of JEB is characterized by blisters, erosions and slow healing wounds only on the </span><span style="font-family: Poppins;">intertriginous skin sites (the folds and bends of the body), the esophagus, and vagina.</span></p><p style="text-align: center;"><span style="font-family: Poppins;"> </span><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b>〰</b></span></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span></p><p><span style="font-family: Poppins;"><b>LOC Syndrome-</b> the protein affected is the Alpha 3 chain of the Laminin 332 gene.</span></p><p><span style="font-family: Poppins;"><b>Common Features: </b></span><span style="font-family: Poppins;"><span style="font-family: Poppins;">formation of blisters on the face
and neck along with the formation of granulation tissue; tooth, eye and
upper airway abnormalities</span> </span></p><p style="text-align: center;"><span style="font-family: Poppins;"> </span><span style="font-family: Poppins;"><span style="font-family: Poppins;"><b>〰</b></span></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span><span style="font-family: Poppins;"><b>〰</b></span></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-84087638388284103752021-01-22T23:01:00.000-05:002021-01-22T23:50:48.764-05:00Kindler Syndrome in Depth <p><b><span style="font-family: Poppins;">Kindler Syndrome is a rare form of Epidermolysis Bullosa due to the lack of production of the protein kindilin-1 in the FERMT1 gene. Kindler syndrome is an autosomal recessive disorder.</span></b></p><p><span style="font-family: Poppins;"> </span></p><p><b><span style="font-family: Poppins;">common characteristics:</span></b></p><ul style="text-align: left;"><li><span style="font-family: Poppins;">usually present at birth</span></li><li><span style="font-family: Poppins;">blisters on the tops of the feet and backs of the hands</span></li><li><span style="font-family: Poppins;">development of paper thin skin and small clusters of blood vessels just under the skin</span></li><li><span style="font-family: Poppins;"> high sensitivity to UV light</span></li><li><span style="font-family: Poppins;">severely fragile mucosa of the eyes, mouth, esophagus, genitals, intestines and urinary tract</span></li><li><span style="font-family: Poppins;">gum disease is common and can lead to tooth loss</span></li><li><span style="font-family: Poppins;">vision can be affected from frequent damage to the eye lids and cornea</span></li><li><span style="font-family: Poppins;">narrow of the esophagus is common</span></li><li><span style="font-family: Poppins;">inflammation to the colon and urethra is common </span></li><li><span style="font-family: Poppins;">an increased risk for squamous cell carcinoma on the skin, lips and oral mucosa.<br /></span></li></ul><p><span style="font-family: Poppins;"><br /></span></p><p><span style="font-family: Poppins;"><br /></span></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-36944863632370921162021-01-22T11:18:00.001-05:002021-01-22T11:52:30.498-05:00EB Simplex in Depth <p style="text-align: justify;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005">Epidermolysis
bullosa (EB) is a rare genetic disorder that causes </span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="ILfuVd"><span class="hgKElc">skin and mucous membrane</span></span>
fragility. It comprises a clinically and genetically heterogeneous group
of disorder characterized by spontaneous or contact/friction–induced
blistering. EB is classified into 4 types–simplex, junctional,
dystrophic, and Kindler syndrome–and 30 sub-types. The disease is caused
by defects in proteins implicated in dermal-epidermal adhesion. To-date, at least
19 genes have been characterized and more than 1000 mutations
identified, thus making it a very diagnosis disorder.</span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"> </span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span style="font-size: medium;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Clinical Classification of EB</span></span></span></span></b></span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><u><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">EBS Suprabasal- </span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Skin fragility syndromes: </span></span></span></span></span></span></span></b></u></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Acral Peeling Skin Syndrome (APSS) [Protein- Transgluminase 5]</span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: c</b></span></span></span></span></span></span></span></span></span></span></span></span></span></span>haracterized by painless peeling of the top layer of skin. The term
"acral" refers to the fact that the skin peeling in this condition is
most apparent on the hands and feet. Occasionally, peeling also occurs
on the arms and legs. The peeling is usually evident from birth,
although the condition can also begin in childhood or later in life.
Skin peeling is made worse by exposure to heat, humidity and other forms
of moisture, and friction. The underlying skin may be temporarily red
and itchy, but it typically heals without scarring. Acral peeling skin
syndrome is not associated with any other health problems.</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">EBS Superficialis (EBSS) [Protein- unknown]</span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features:</b></span></span></span></span></span></span></span></span></span></span></span></span></span></span> milia and atrophic (</span></span><span style="font-family: Poppins;"><span style="color: #073763;"><span class="ILfuVd"><span class="hgKElc">indented)</span></span> scarring, as well as involvement of oral and/or ocular surfaces. the presence of blisters and the absence of spontaneous continual exfoliation or peeling.</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"></span></span><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Lethal Acantholytic EBS (EBS- acanth) [Protein- Desmoplaskin, Plakoglobin]</span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span>characterized by
generalized oozing erosions, usually in the absence of blisters.</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Desmoplaskin Woolly Hair Syndrome [Protein- Desmoplaskin] </span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></b><br /><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span>hair that is unusually coarse, dry,
fine, and tightly curled. In some cases, the hair is also sparse. The
woolly hair texture typically affects only scalp hair and is present
from birth. Starting early in life, affected individuals also develop
palmoplantar keratoderma, a condition that causes skin on the palms of
the hands and the soles of the feet to become thick, scaly, and
calloused; </span></span></span></span></span></span></span>Cardiomyopathy, which is a disease of <a class="image-modal" data-alt="A cross-section of various structures that make up the normal heart." data-caption="" data-credit="U.S. National Library of Medicine" data-filepath="images/PX0002LE_PRESENTATION.jpeg" data-imgtype="genetics" data-pix="PX0002LE" data-sourceurl="https://www.nlm.nih.gov/" data-title="Normal heart anatomy" href="https://medlineplus.gov/images/PX0002LE_PRESENTATION.jpeg" id="PX0002LE_1" title="Show image">the heart muscle</a>,
is a life-threatening health problem that can develop in people with
keratoderma with woolly hair. Unlike the other features of this
condition, signs and symptoms of cardiomyopathy may not appear until
adolescence or later. Complications of cardiomyopathy can include an
abnormal heartbeat (arrhythmia), heart failure, and sudden death.</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Plakoglobin Deficiency [Protein - </span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Plakoglobin]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">mild skin involvement, </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="ILfuVd"><span class="hgKElc">thickening of the skin on the palms of the hands and soles of the feet</span></span> and arrhythmogenic heart disease.</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Ectodermal Dysplasia Syndrome [Protein Plakophilin 1]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span><span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><u>manifests with </u></span><u><a href="https://www.sciencedirect.com/topics/medicine-and-dentistry/skin-fragility" title="Learn more about Skin Fragility from ScienceDirect's AI-generated Topic Pages">skin fragility</a><span>, <a href="https://www.sciencedirect.com/topics/medicine-and-dentistry/palmoplantar-keratoderma" title="Learn more about Palmoplantar Keratoderma from ScienceDirect's AI-generated Topic Pages">palmoplantar keratoderma</a><span>, abnormal hair growth, and <a href="https://www.sciencedirect.com/topics/medicine-and-dentistry/nail-dystrophy" title="Learn more about Nail Dystrophy from ScienceDirect's AI-generated Topic Pages">nail dystrophy</a> and, in some but not all cases, with defective sweating</span></span></u></span><u> <br /></u></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><u><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></u></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><u><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">EBS Basal- More common type of EBS</span></span></span></span></span></span></span></span></span></span></span></span></span></span></u></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Localized EBS (EBS-loc) <b>[Protein- Keratin 5 or Keratin 14]</b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035">most
common form; rarely present at birth and usually appear when the child begins to crawl or walk; characterized by blisters confined to the palms
and soles and, in some cases, by oral erosions or blisters during
infancy. Lesions can be present in any part of the body as a result of
skin trauma.</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Generalized Intermediate EBS (EBS-gen intermed) </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">[Protein- Keratin 5 or Keratin 14]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">common features: </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">usually present at birth or within the first days of life; p</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035">resents with non-herpetiform blisters (not in clusters); anemia and growth retardation are uncommon in this subtype.</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Generalized Severe EBS (EBS- gen sev) </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">[Protein- Keratin 5 or Keratin 14]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><br /></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">common features: </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035">a
less frequent subtype; present at birth with widespread skin loss; usually associated with marked morbidity and
mortality during the neonatal period or early infancy. Its most
distinctive feature is the formation of intact blisters grouped in an
arch-shaped or clustered distribution (herpetiform). Patients with this form usually
develop skin thickening on the palms and soles, giving rise to keratosis
that may resolve on reaching adulthood. Some patients have nail
distrophy atrophic scarring, milia, mucosal involvement (laryngeal
stenosis), anemia, and delayed growth. </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">EBS with Mottled Pigmentation (EBS-MP) </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">[Protein- Keratin 5]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">common features: </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">blistering may begin at birth; have a mottled appearance of the skin; may bruise more easily and the skin may seem to age more quickly. </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Migratory Circinate EBS (EBS-migr) </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">[Protein- Keratin 5]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">common features: </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">appears to be phenotypically milder than
EBS-generalized severe; is characterized by belt-like areas of
erythema with multiple vesicles and small blisters at the advancing edge
of erythema. Lesions appear from birth primarily on hands, feet and
legs. Nails and mucosa were not affected. The lesions healed with brown
pigmentation, but without scarring. </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">KLHL24 EBS</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> [Protein-Keratin 14] </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">common feature: </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">wide spread blistering can occur; </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><br /><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"><b>alopecia </b>(</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"><span class="ILfuVd"><span class="hgKElc">a condition that causes hair to fall out in small patches</span></span>); <b>follicular and cutaneous atrophy</b> (reduced hair follicles and </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><br /><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"><span class="ILfuVd"><span class="hgKElc">sebaceous glands) and most significantly- </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><br /><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"><span class="ILfuVd"><span class="hgKElc"><span class="ILfuVd"><span class="hgKElc"><b>Dilated cardiomyopathy</b>- a condition in which the heart becomes enlarged and cannot pump
blood effectively. Symptoms vary from none to feeling tired, leg
swelling, and shortness of breath. It may also result in chest pain or
fainting.</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"><span class="ILfuVd"><span class="hgKElc"><span class="ILfuVd"><span class="hgKElc"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="sec0035"><span class="ILfuVd"><span class="hgKElc"><span class="ILfuVd"><span class="hgKElc"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">K14 Recessive EBS (EBS-AR) </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">[Protein- Keratin 14]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">common features: </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">characterized by generalized or, less frequently, localized acral (hands, feet, fingers, toes) blistering.</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><b><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">EBS with Muscular Dystrophy (EBS-MD) [Protein- Plectin 1]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">generalized blistering associated with muscular dystrophy.<b> </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b> </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b> </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">EBS with Pyloric Atresia (EBS-PA) [Protein- Plectin, Alpha4Beta6]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span>generalized severe blistering with widespread congenital absence of skin and pyloric atresia.</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b> </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Ogna EBS (EBS-Og) [Protein- Plectin]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span>characterized by sometimes widespread, primarily acral blistering.</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b> </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p><b><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030">Recessive EBS BP230 Deficiency (EBS-AR BP230) [Protein- antigen-1 BP230]</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></b></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span>mild, predominantly acral, trauma-induced skin fragility, resulting in
blisters. Blisters mostly affect the feet, including the dorsal side,
and are often several centimeters big.</span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b> </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>Recessive EBS Exophilin 5 Deficiency (EBS-AR exo 5) [Protein Exophilin 5]</b><br /></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><b>common features: </b></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span>mild, generalized trauma-induced scale crusts and intermittent
blistering, sometimes combined with erosions and bleeding, recovering
with slight scarring and post-inflammatory hyperpigmentation. Clinical
symptoms improve with age.</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span style="font-family: Poppins;"><span style="color: #073763;">〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span><span style="font-family: Poppins;"><span style="color: #073763;"> 〰</span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span style="font-family: Poppins;"><span style="color: #073763;"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p><p style="text-align: center;"><span style="font-family: Poppins;"><span style="color: #073763;"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span style="font-family: Poppins;"><span style="color: #073763;">©Garrett's House 2021 </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSection elsevierViewall" id="abst0005"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"><span class="elsevierStyleSections"><span class="elsevierStyleSection elsevierViewall" id="sec0025"><span class="elsevierStyleSection elsevierViewall" id="sec0030"> </span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></span></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-24753299342139268642021-01-14T14:12:00.001-05:002021-01-14T14:19:15.838-05:00<div dir="ltr" style="text-align: left;" trbidi="on">
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Infections and EB Due to constantly having open wounds, those with EB are highly susceptible to getting an infection. </div>
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There are 5 main types of infection those with EB get: </div>
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Staph </div>
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Strep </div>
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Pseudomonas </div>
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MRSA - (Methicillin-resistant Staphylococcus Aureus)</div>
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Yeast </div>
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(there
are also dozens of other bacterial infection that one can contract, but
these seem to be most common among EB) signs of an infection</div>
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~ red or swollen wound, sometimes it will feel hot</div>
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~foul odor</div>
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~excessive drainage, more than usual.</div>
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<i>(Greenish-blue
drainage can indicate a pseudomonas infection. While cloudy or
yellowish drainage can indicate a step or staph infection</i>. )<b><br />
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~a fever </div>
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Only a wound culture performed by a doctor can tell you what type of bacteria is causing the infection. </div>
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Typically
skin infections are treated with topical antibiotics, such as
Polysporin or Bacitracin. Sometimes a prescription strength topical
antibiotic such as Bactroban is necessary. Only use Bactroban as
prescribed. Over use of it can cause your body to become resistant to
it and it will no longer work when you really need it too. The same
with oral antibiotics. Use those only when necessary so that your body
doesn't become resistant to those as well. </div>
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In addition to topical and oral antibiotics, there are other home remedies that you can do to help eliminate the bacteria. </div>
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For Pseudomonas Infection, you will want to use an acetic solution and the most common thing to use is Vinegar. </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<b>The acetic acid formula is: </b></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
Mix 1 ounce of 5% vinegar in 19 ounces water. </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
Vinegar
baths or compress can be used on any age. Just make sure you rinse
well with clear, clean water after a Vinegar bath, otherwise excess
itching and irritation may occur. </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
For
a Staph or Strep related infection, (including MRSA) you can take
bleach bath or apply bleach compresses to the infection area to help
kill the bacteria.<br />
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<span style="font-size: medium;"><br />
</span></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<b><span style="font-size: x-small;">Bleach Bath Formula for Adults and Children OVER the age of 1: </span></b></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
Mix 1 teaspoon bleach in 1 gallon water.</div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<b>For Children and Infants </b><b>UNDER the age of 1, </b><br />
<br />
Mix 1/2 teaspoon bleach or less to 1 gallon of water.<br />
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
No
matter what age, make sure you rinse well with clear, clean water after a
bleach bath, otherwise excessive itching and irritation may occur. I
have heard that many with EB take bleach or vinegar baths daily to help
keep infections at bay. And found them to be very effective.<br />
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<b>NEVER</b>
mix Vinegar and Bleach together in the same bath. A chemical reaction
can occur and produce a toxic fume causing respiratory issues. In
addition, adding other agents such as soap, salt, or bubble bath can
alter the PH levels of the bleach (or vinegar) solution making it less
effective against the bacteria. It's also important to mix the correct
amount of water and bleach (or vinegar) together. Too much water can
dilute the bleach (or vinegar) making it less effective while too much
bleach (or vinegar) can cause stinging upon contact with the open wounds</div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
Unfortunately,
infections tend to be apart of having EB. Prevention in the best
thing, and to recognize an infection quickly so that prompt treatment
can begin.<br />
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<span style="font-size: medium;"><b>Suggestions for the entire family when dealing with EB and recurrent infections.</b> </span><br />
<br />
-swab all family members noses with prescription bactraban to help stop
the spread between family members. Most people carry the staph bacteria
on their skin and in their nose and don't even know it. It usually
doesn't cause a problem until it gets into an open wound. </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
- NEVER share towels, blankets, sheets, etc... </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
-wash bed liens in hot water daily of the person with an active infection </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
-wash your hand A LOT </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
-for
children in school, once they get home from school/day care have them
wash their hands and change their clothes. You wouldn't believe the
kind of bacteria and germs that can come home from school </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
-change your clothes and wash your hands after you return home from work or a doctors appointment. </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
-if possible do the same for the person in the family with EB after returning from the doctors or hospital. </div>
<div style="font-family: "Trebuchet MS", sans-serif;">
<br /></div>
<div style="font-family: "Trebuchet MS", sans-serif;">
-disinfect toys of small children frequently to prevent the spread of bacteria.<br />
<br />
<br />
<br />
<b><span style="font-size: medium;"> Other Tips on Infections:</span></b><br />
<br />
*if dealing with MRSA, there is a product you can get from your doctor
to add to the bath water (and when washing clothing) called <b style="font-weight: normal;">chlorhexidine (Hibiclens)to help fight the infection. </b><br />
<br />
<b style="font-weight: normal;">*Medical Grade Honey and products with
Silver in them also help fight many bacterial infections. It is always
best to check with your doctor before trying something new as some over
the counter products can interfere with prescription medical (either
oral or topical) </b></div><div style="font-family: "Trebuchet MS", sans-serif;"><b style="font-weight: normal;"> </b></div><div style="font-family: "Trebuchet MS", sans-serif;"><b style="font-weight: normal;"> </b></div><div style="font-family: "Trebuchet MS", sans-serif; text-align: center;"><b style="font-weight: normal;"> </b></div><div style="font-family: "Trebuchet MS", sans-serif; text-align: center;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span> </div>
</div>
Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-48622032264954153172021-01-14T13:47:00.004-05:002021-01-14T14:19:49.352-05:00EB Statistics <p> </p><div style="font-family: Verdana, sans-serif; text-align: center;"><span style="font-size: x-large;"><b>EB Statistics </b></span></div><div style="font-family: Verdana, sans-serif; text-align: center;"><span style="font-size: small;"><br />
</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">The
incidence column is the one that estimates number born in a given
year. The last official birthrate numbers are form 2009, and that year
it was <span style="color: black; line-height: 115%;">4,130,665 (2010 and 2011 are not available)</span></span></div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">Material is based on the NEBR study population </span></div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><br />
</div><table border="0" cellpadding="0" cellspacing="0" class="nobrd" style="font-family: Verdana, sans-serif;"><tbody>
<tr> <td><span style="font-size: small;">EB type or subtype:</span></td> <td><span style="font-size: small;">Prevalence* </span></td> <td><span style="font-size: small;">Incidence**</span></td><td><br /></td><td><br /></td><td><br /></td><td><br /></td><td><br /></td><td><br /></td><td><br /></td><td><br /></td><td><br /></td></tr>
<tr> <td><span style="font-size: small;">EB (All types and subtypes, including unclassified patients)</span></td> <td style="text-align: center;"><span style="font-size: small;">8.22</span></td> <td style="text-align: center;"><span style="font-size: small;">19.60</span></td></tr>
<tr> <td><span style="font-size: small;">EBS</span></td> <td style="text-align: center;"><span style="font-size: small;">4.60</span></td> <td style="text-align: center;"><span style="font-size: small;">10.75 </span></td></tr>
<tr> <td style="padding-left: 20px;"><span style="font-size: small;">EBS, Localized (formerly Weber- Cockayne)</span></td> <td style="text-align: center;"><span style="font-size: small;">3.14</span></td> <td style="text-align: center;"><span style="font-size: small;">6.81</span></td></tr>
<tr> <td style="padding-left: 20px;"><span style="font-size: small;">EBS, all others</span></td> <td style="text-align: center;"><span style="font-size: small;">1.46</span></td> <td style="text-align: center;"><span style="font-size: small;">3.95</span></td></tr>
<tr> <td><span style="font-size: small;">JEB</span></td> <td style="text-align: center;"><span style="font-size: small;">0.44 </span></td> <td style="text-align: center;"><span style="font-size: small;">2.40 </span></td></tr>
<tr> <td style="padding-left: 20px;"><span style="font-size: small;">JEB-Herlitz</span></td> <td style="text-align: center;"><span style="font-size: small;">0.07</span></td> <td style="text-align: center;"><span style="font-size: small;"><0.41</span></td></tr>
<tr> <td style="padding-left: 20px;"><span style="font-size: small;">JEB, other</span></td> <td style="text-align: center;"><span style="font-size: small;">0.37</span></td> <td style="text-align: center;"><span style="font-size: small;"><2.04</span></td></tr>
<tr> <td><span style="font-size: small;">DDEB</span></td> <td style="text-align: center;"><span style="font-size: small;">0.99 </span></td> <td style="text-align: center;"><span style="font-size: small;">2.86</span></td></tr>
<tr> <td><span style="font-size: small;">RDEB</span></td> <td style="text-align: center;"><span style="font-size: small;">0.92 </span></td> <td style="text-align: center;"><span style="font-size: small;">2.04</span></td></tr>
<tr> <td style="padding-left: 20px;"><span style="font-size: small;">RDEB, severe generalized (formerly Hallopeau- Siemens)</span></td> <td style="text-align: center;"><span style="font-size: small;">0.42</span></td> <td style="text-align: center;"><span style="font-size: small;">0.41</span></td></tr>
<tr> <td style="padding-left: 20px;"><span style="font-size: small;">RDEB, all others</span></td> <td style="text-align: center;"><span style="font-size: small;">0.49</span></td> <td style="text-align: center;"><span style="font-size: small;">1.63</span></td></tr>
</tbody></table><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">*Prevalence of EB patients per one million births (1990) </span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">**Incidence of EB births per one million live births (1986-1990) </span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">Fine,
Jo-David and Hintner, Helmut. (2008) "Life with Epidermolysis Bullosa
(EB): Etilogy, Diagnosis, Multidisciplinary Care and Therapy"
Springer-Verlag/Wien, New York. </span></div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">The
number in the Incidence Column is per 1 million births so to find the
actual number of new cases of EB each year in the US, multiple that
number by 4.13 (the approximate total number of births in the US each
year)</span></div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">So there is approximately 80 new cases of EB in the US each year. Of those 80- </span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;"><br />
</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">45 will have some form of EB Simplex</span></div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">10 will have some form of Junctional</span></div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">12 will have Dominant Dystrophic EB</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;"><br />
</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">10 will have Recessive Dystrophic EB</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;"><br />
</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;">This is just an approximate # and some years there will be more or less in each category. </span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;"><br />
</span></div><div style="font-family: Verdana, sans-serif;"><span style="font-size: small;"><br />
</span></div><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-yNx9KBOR93Y/X-BkKRVBi-I/AAAAAAAAijs/Sl1FOYlIjwoddcvwc93VaSR829kr457cwCLcBGAsYHQ/s720/131090510_1570983426441696_1713494582077262477_n.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="720" data-original-width="720" height="320" src="https://1.bp.blogspot.com/-yNx9KBOR93Y/X-BkKRVBi-I/AAAAAAAAijs/Sl1FOYlIjwoddcvwc93VaSR829kr457cwCLcBGAsYHQ/s320/131090510_1570983426441696_1713494582077262477_n.jpg" /></a></div><br /><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif;"><br />
</div><div style="font-family: Verdana, sans-serif; text-align: center;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span>
</div>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-74571933669051332382021-01-01T07:15:00.002-05:002021-01-14T14:23:45.462-05:00Survey Results<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-size: medium;"><span style="font-family: verdana;"><span><span>In the fall of 2008 I started a survey among those with EB or who had children with EB to get an idea of when kids with EB, walked, crawled, and other milestone that all kids experience. I kept adding data as people were willing to share. As of March 2009, apx 75 patients participated in the survey. Here are the results. </span><br />
<br />
<br />
<b><br />
</b><span><b>The average age for EB as a whole: </b><br />
<br />
Rolled over: 6-7months <br />
<br />
Sat unassisted: 7-8 months <br />
<br />
Crawled: 11 months <br />
<br />
Pulled to a standing position: 13-14 months <br />
<br />
Walked: 23-24 months <br />
<br />
Got 1st tooth: 8 1/2 months <br />
<br />
Began sleeping through the night on a regular bases: 18-19 months <br />
<br />
Length of hospital stay after birth: 28.1 days <br />
<br />
Received some type of therapy (OT, PT, speech,feeding): 51% of those survey did <br />
<br />
Found it to be helpful: of those 51%, 28% found therapy to be helpful <br />
<br />
Suffer from reflux: 69% yes; 31% no<br />
<br />
Are lactose intolerant: 18% yes; 72% no<br />
<br />
Have/had a G-tube: 32% yes; 68% no<br />
<br />
Age they received a g-tube: 20.4 months <br />
<br />
Issues with constipation: 59% - yes; 41% no<br />
</span></span></span></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-size: medium;"><span style="font-family: verdana;"><span><span> <br />
🔆🔆🔆🔆🔆🔆🔆🔆🔆🔆🔆🔆🔆🔆</span></span></span></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-size: medium;"><span style="font-family: verdana;"><span><span><br />I re-did the survey in 2020 and these were the results: </span></span></span></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-size: medium;"><span style="font-family: verdana;"><span><span></span></span></span></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-size: medium;"><span style="font-family: verdana;"><br /><span><span><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 a8c37x1j keod5gw0 nxhoafnm aigsh9s9 d3f4x2em fe6kdd0r mau55g9w c8b282yb iv3no6db jq4qci2q a3bd9o3v knj5qynh oo9gr5id" dir="auto"><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"><br /></span></div></span></span></span></span></span><div style="text-align: center;"><div style="text-align: center;"><span style="font-size: medium;"><span style="font-family: verdana;"><b><span><span><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 a8c37x1j keod5gw0 nxhoafnm aigsh9s9 d3f4x2em fe6kdd0r mau55g9w c8b282yb iv3no6db jq4qci2q a3bd9o3v knj5qynh oo9gr5id" dir="auto"><div class="bi6gxh9e" style="text-align: left;"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">EB Development Survey Results:</span></div></span></span></span></b></span></span></div><span style="font-size: medium;"><span style="font-family: verdana;"><span><span><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 a8c37x1j keod5gw0 nxhoafnm aigsh9s9 d3f4x2em fe6kdd0r mau55g9w c8b282yb iv3no6db jq4qci2q a3bd9o3v knj5qynh oo9gr5id" dir="auto"><div class="bi6gxh9e" style="text-align: left;"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div></span></span></span></span></span></div><span style="font-size: medium;"><span style="font-family: verdana;"><span><span><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 a8c37x1j keod5gw0 nxhoafnm aigsh9s9 d3f4x2em fe6kdd0r mau55g9w c8b282yb iv3no6db jq4qci2q a3bd9o3v knj5qynh oo9gr5id" dir="auto"><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">63 total participants</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">42 have EBS</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">9 have RDEB</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">9 have DDEB</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">2 have JEB</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">1 has an unknown form of EB</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Reflux:</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">60% no</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">40% yes</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Gtube:</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">90% no</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">10% yes</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Constipation:</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">46% no</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">54% yes</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Received OT, PT or other therapy:</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">47% no</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">52% yes</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Of the 52%</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Did you find it beneficial?</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">57% yes</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">25% no</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">19% maybe</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Lactose Intolerant?</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">90% no</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">10% yes</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Average Age in months for:</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Sleeping thru the night: 14 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">NICU stay after birth: 13 days</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">First Tooth: 8 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">First words: 16 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Walking: 20 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Standing up: 15 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Crawling: 11 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Sitting unassisted: 7.4 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">Rolled over: 5.5 months</span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id"> </span></div><div class="bi6gxh9e"><span class="d2edcug0 hpfvmrgz qv66sw1b c1et5uql rrkovp55 jq4qci2q a3bd9o3v knj5qynh oo9gr5id">I
was hoping to break each item down by type of EB, but not everyone
answered with their type/subtype, so I wasn't able to get an accurate
breakdown. Some of the results did surprise me though- particularly the
reflux one. Perhaps I should have listed it as silent reflux. But no
matter, these are interesting none the less. Thank you those who
participated <span class="pq6dq46d tbxw36s4 knj5qynh kvgmc6g5 ditlmg2l oygrvhab nvdbi5me sf5mxxl7 gl3lb2sf hhz5lgdu"><img alt="🙂" height="16" src="https://static.xx.fbcdn.net/images/emoji.php/v9/t4c/1/16/1f642.png" width="16" /></span></span></div></span>
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Just like with healthy children, all EB children are different and some will do things sooner or later than the average child with EB. I hope this will give parents are idea of when they might expect their child to reach a certain developmental milestone and not to worry if their child is a little bit behind other kids their age. </span></span></span></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-size: medium;"><span style="font-family: verdana;"><span><span> </span></span></span></span></div><div style="text-align: center;" trbidi="on"><span style="font-size: medium;"><span style="font-family: verdana;"><span><span><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span> <br /></span></span></span></span></div><span style="font-size: medium;"><span style="font-family: verdana;">
</span></span>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-31558466400599393962015-10-28T08:00:00.001-04:002021-01-14T14:23:56.417-05:00The Proof it works!!<div dir="ltr" style="text-align: left;" trbidi="on">
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The proof blended diet works! </div>
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We started blended diet around age 2. At the time he has been on formula since birth. At 18 months he weighted 24 pounds (50%) and was 32" (50%) tall and his EB was still very severe; about 60% of his body had open wounds. We had blood work on in the fall of 2012 and here were the results <br />
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<b>Vit D 26 </b>(range 30-80)<br />
<b>Creatinine 0.52</b> (range 0.26-0.42)<br />
<b>Protein 6.4</b> (range 6.5-8)<br />
<b>Sed Rate 91</b> (range 0-15)<br />
<b>Iron 38</b> (range 45-182)<br />
<b>Hemoglobin 9.3 </b>(range 9.4-14.3<br />
<b>Hemaoticrit 28.3</b> (range 34-40)<br />
Calcium 8.9 (range 8.5-10.5)<br />
<b>Ferritin 25</b> (range 36-84)<br />
<b>Zinc 54</b> (range 60-120)<br />
Selenium 80 (range 23-190)<br />
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<b><i>(items in bold are abnormal results) </i></b><br />
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My request for yearly blood work went ignored because no lab was willing to draw blood due to his EB.<b><i> </i></b>Finally last month we were able to get a lab to draw some labs. At age 4 1/2 he is 39 pounds (75%) and 43" (95%) tall. His skin looks amazing and if he wasn't so rough when he played, we could stop wrapping all together but he still needs the protection! After 2 1/2 years on blended diet this is the the blood work results<b><i>.</i></b><br />
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Vit D 30 (range 30-80)<br />
Creatinine 0.30 (range 0.26-0.42)<br />
Protein 7.5 (range 6.5-8)<br />
Sed Rate 7 (range 0-15)<br />
<b>Iron 42</b> (range 45-182)<br />
Hemoglobin 11.9 (range 9.4-14.3)<br />
Hemaoticrit 34.3 (range 34-40)<br />
Calcium 9.8 (range 8.5-10.5)<br />
<b>Ferritin 30</b> (range 36-84)<br />
Zinc 62 (range 60-120)<br />
Selenium 134 (range 23-190)<br />
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<b><i>(items in bold are abnormal results) </i></b><br />
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Low Iron and Ferritin are pretty normal in EB. It may also be because GI took him off his multivitamin back in March when he turned 4 and they said he was too old for the infant version but offered no alternative<i><b>. </b></i>So we have started a new multi-vitamin with iron and added more iron rich foods to his blended diet<i><b> </b></i>and will recheck the results in December. <b><i></i></b><br />
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In addition to blood work, you can also see the difference in his skin!! <br />
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Joey's back before blended diet, </div>
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a couple months after starting the blended diet, </div>
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a year after </div>
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and his back today. </div>
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In
addition to improved blood work and wound healing, blended diet also
has eliminated his constipation, reduced his over all inflammation
levels and a decrease in his dosage of acid-reflux medication.<br />
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I do understand that blended diet my not be the answer for everyone with a G-tube but I think the results speak for themselves. I can only imagine how bad his skin would look and how bad his results would be if we was only getting sugar supplements all day long<i><b>. </b></i>He hasn't had a skin infection in year<i><b>s </b></i>and the number is colds/viruses he gets yearly have decreased each year as well.<i><b> </b></i>His doctors are very pleased with his overall heath<i><b>. </b></i>There is no plan to remove his G-Tube anytime soon. He can and does eat by mouth but not enough to survived without his tube and that is OK! If he goes to college with it, I don't care. As long as it isn't causing him any issues, the benefits we are seeing certainly out weigh the risks.<br />
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<span style="font-size: x-small;"><span face="Verdana,sans-serif"><b><span style="font-size: xx-small;"><i><span face="Verdana,sans-serif"><span style="color: black;">*Disclaimer:
I am not a medical professional. This is not medical advice. i am
just sharing what I have learned along the way with the hope it makes
things easier for others. </span></span></i></span></b></span></span>
<span style="font-size: x-small;"><br /></span></div><div dir="ltr" style="text-align: left;" trbidi="on"></div><div style="text-align: center;" trbidi="on"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span><br /></div><div dir="ltr" style="text-align: left;" trbidi="on">
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Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-85502789571385430592015-10-27T22:04:00.001-04:002021-01-14T14:24:08.015-05:00Administering Blended Diets and Recipes! <div dir="ltr" style="text-align: left;" trbidi="on">
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Administering blended diets is a little different than feeding formula. </div>
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Most blended have to be pushed by hand quickly vs using a feeding pump over the course of an hour. </div>
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<a href="https://lh3.googleusercontent.com/-_ATBZy945N0/Vi_JGcb58AI/AAAAAAAAGE4/mFj5JHNSTiI/s1600/20151027_113255.jpg" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="179" src="http://lh3.googleusercontent.com/-_ATBZy945N0/Vi_JGcb58AI/AAAAAAAAGE4/mFj5JHNSTiI/s320/20151027_113255.jpg" width="320" /></a>We use the wider extension (blous) to attach to the mic-key button. And we use a 60ml syringe to push the blends into the stomach. We push it thru very quickly because that is what my son prefers. But some kids need it pushed much slower. It will also depend on your child and what they prefer and can tolerate. Once the feeding is finished, we flush the line with 20cc of water. Water is very important for kids with eb to keep the kidneys healthy. The amount of water each kid needs depends on their age, weight, and health. Your child's doctor can tell you how much free what they need daily. </div>
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We feed at normal meal/snack times, to mimic when a child without a gtube would eat. We don't do overnight feeds, which is common in kids with gtubes. The human body isn't designed to be feed continuously overnight. The gut needs time to rest and when one is feed overnight it really never has time to rest. </div>
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Blended diet is time consuming and not as easy as using formula. But the health benefit it SO worth all the extra work. </div>
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Here are some example recipes we use:</div>
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Breakfast/Snack</div>
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Lunch </div>
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<a href="http://3.bp.blogspot.com/-7elBEcU8Vq4/Vi-3pt8J-AI/AAAAAAAAGEk/UntxK65QdtQ/s1600/lunch.JPG" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" src="http://3.bp.blogspot.com/-7elBEcU8Vq4/Vi-3pt8J-AI/AAAAAAAAGEk/UntxK65QdtQ/s1600/lunch.JPG" /></a> </div>
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Dinner</div>
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<a href="http://2.bp.blogspot.com/-U9bMZUYJa58/Vi-3pvW4QkI/AAAAAAAAGEg/8Lt2fUxQBgM/s1600/dinner.JPG" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" src="http://2.bp.blogspot.com/-U9bMZUYJa58/Vi-3pvW4QkI/AAAAAAAAGEg/8Lt2fUxQBgM/s1600/dinner.JPG" /></a></div>
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Each one is what I call a "batch". I then divide each batch into a day's worth of blends. It is at that point I add other things; oils, the baby oatmeal, Benacalorie, etc... Then I divide all that into a service size for my son, place in snack size Zip-Loc bag and freeze until we need them. I typically blend once a week making enough for 7-10 days worth of meals.<br />
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You can do any combination of food you'd like. It all depends on your child's needs, food allergies, etc... These are just the things we have tried.<br />
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<span style="font-size: x-small;"><span face="Verdana,sans-serif"><b><span style="font-size: xx-small;"><i><span face="Verdana,sans-serif"><span style="color: black;">*Disclaimer:
I am not a medical professional. This is not medical advice. i am
just sharing what I have learned along the way with the hope it makes
things easier for others. </span></span></i></span></b></span></span>
<span style="font-size: x-small;"></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-size: x-small;"><br /></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><br /></div><div style="text-align: center;" trbidi="on"><span style="font-size: x-small;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span></span></div>
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Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-90507991659203036032015-10-26T21:50:00.006-04:002021-01-14T14:24:30.772-05:00Blended Diet Ingredients<div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="font-size: small;"><span face="Verdana,sans-serif">Once you have read <a href="http://ingarrettsmemory.blogspot.com/2015/10/blended-diet-basics.html" target="_blank">blended diet basics</a>, it is now time to move into creating recipes! </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">While those with EB require more calories each day due to wound healing, you need to look at more than just calories when planning a blended diet. Protein, iron, zinc, calcium vitamin D are just a few things that are also vital for a healthy body. </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">With blended diet your goal is to create a well rounded meal. </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><br /></span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">First make a list of any foods your child is allergic too. Or any foods your child needs to avoid. Kids with EB should avoid foods that are highly acidic to avoid triggering their acid reflux. They also need diets high in protein, zinc and iron for optimal wound healing. </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Next make lists of the major food groups: </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Fruits</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Veggies</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Protein</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Oils </span></span><br />
<span style="font-size: small;"><span face="Verdana,sans-serif">Liquids</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">You can choose whatever foods you want to try. But as an example I will share the lists of foods we have tried along with the nutritional values of those foods. I also rank them by calorie count. I know the pictures are a little fuzzy. It was hard to turn an Excel spreed sheet document into a picture. Clicking on them might help or you can email me and I'd be happy to send the Excel spreed sheet to you. sara.denslaw@gmail.com</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><a href="http://2.bp.blogspot.com/-2Zcul8BH1sk/Vi2HEr5liNI/AAAAAAAAGAw/2zFO-GSAq9Y/s1600/fruit.JPG" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="140" src="http://2.bp.blogspot.com/-2Zcul8BH1sk/Vi2HEr5liNI/AAAAAAAAGAw/2zFO-GSAq9Y/s640/fruit.JPG" width="640" /></a></span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><a href="http://3.bp.blogspot.com/-UWnfkHP5Xek/Vi2HFMYOChI/AAAAAAAAGBM/cN7KuHv3TKo/s1600/protein.JPG" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="150" src="http://3.bp.blogspot.com/-UWnfkHP5Xek/Vi2HFMYOChI/AAAAAAAAGBM/cN7KuHv3TKo/s640/protein.JPG" width="640" /></a></span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><a href="http://4.bp.blogspot.com/-aFpLjSPzYIc/Vi7TulrarnI/AAAAAAAAGCw/xqvEqaADkww/s1600/veggies2.JPG" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="156" src="http://4.bp.blogspot.com/-aFpLjSPzYIc/Vi7TulrarnI/AAAAAAAAGCw/xqvEqaADkww/s640/veggies2.JPG" width="640" /></a></span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><a href="http://3.bp.blogspot.com/-ggocZQZ-pFI/Vi2HE9T9o6I/AAAAAAAAGBQ/SVgJ7PBMRys/s1600/oils.JPG" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="150" src="http://3.bp.blogspot.com/-ggocZQZ-pFI/Vi2HE9T9o6I/AAAAAAAAGBQ/SVgJ7PBMRys/s640/oils.JPG" width="640" /></a></span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><b>Making Blends</b></span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">I choose one or two items from each food group, mixing and matching each time to create a wide variety of blends. I go off what is available in stores and what is on sale. I always pick fresh over frozen and frozen over canned. Avoid canned fruits and veggies (except canned pumpkin) when possible. Canned fruits and veggies tend to be high in sodium and preservatives. The oils are to add calories since most fruits and veggies aren't high in calories. Some oils are very beneficial to the skin and over all health. And with 120 calories per tablespoon is a great way to add calories without adding a ton of volume. </span></span><br />
<span style="font-size: small;"><span face="Verdana,sans-serif"><br /></span></span>
<span style="font-size: small;"><span face="Verdana,sans-serif"><br />I create blends in large batches, and then divide them into 30oz portions. To EACH 30oz portion I add the following:</span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">1) 3 tablespoons <span style="color: black;">Happy Bellies Organic Baby Oatmeal. )The servicing size is 3
tablespoon and it provides 80% of the RDA of iron. Plus it had
probiotics in it as well.)</span></span></span><br />
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<a href="https://lh3.googleusercontent.com/-ZJB03iWSNPM/Vi5cIBfz9QI/AAAAAAAAGCU/CL_tPjcVwVc/s1600/20151026_125845.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://lh3.googleusercontent.com/-ZJB03iWSNPM/Vi5cIBfz9QI/AAAAAAAAGCU/CL_tPjcVwVc/s320/20151026_125845.jpg" width="180" /></a><a href="https://lh3.googleusercontent.com/-Cx8iucGAE4c/Vi5cGtt3KyI/AAAAAAAAGCM/UoyjDzK02Pg/s1600/20151026_125905.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="http://lh3.googleusercontent.com/-Cx8iucGAE4c/Vi5cGtt3KyI/AAAAAAAAGCM/UoyjDzK02Pg/s320/20151026_125905.jpg" width="180" /></a></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><span style="color: black;">2) 1 packet of Carnation Instant Breakfast</span></span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif"><span style="color: black;">3) 1 tray of </span><a href="https://www.nestlehealthscience.us/brands/benecalorie/benecalorie" target="_blank">Benecalorie</a></span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">Once all that is mixed, I then divide the blend into 6oz serving and freeze them in snack size Zip-Loc bags. You can store blends in the freezer for months but only in the fridge for 2-3 days. I take out baggies as we need them and thaw! </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">The <span style="color: black;"><a href="http://ndb.nal.usda.gov/ndb/foods" target="_blank">National Nutrient Database </a>is what I used to determine the nutritional value of each food item. You can look up ANY item you want to use to determine the break down of the nutritional value. </span></span></span><br />
<br />
<span style="font-size: small;"><span face="Verdana,sans-serif"><span style="color: black;">This is just a basic idea of the MANY wants to create a blended diet. Tomorrow I will share some of the recipes we use to give you even more ideas on how to create a blended diet for your child! </span></span></span><br />
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<b><span style="font-size: x-small;"><i><span face="Verdana,sans-serif"><span style="color: black;">*Disclaimer: I am not a medical professional. This is not medical advice. i am just sharing what I have learned along the way with the hope it makes things easier for others. </span></span></i></span></b> </div>
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<span style="font-size: small;"><span face="Verdana,sans-serif"><span style="color: black;"><br /></span></span></span></div><div dir="ltr">
<br /></div><div style="text-align: center;"><span style="font-size: small;"><span face="Verdana,sans-serif"><span style="color: black;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span></span></span></span></div>
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Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-13896270732771903082015-10-26T21:50:00.005-04:002021-01-14T14:24:17.828-05:00Blended Diet Basics <div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-size: small;"><span face="Verdana,sans-serif">There are many things you need to figure out before you start down the blended diet path. </span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">First you need to determine how many ounces per day your child needs. Your doctor or nutritionist is the best person to get that number from. Next you need to determine how many ounces per serving your child can tolerate at a time. Then once you figure that out you can come up with a feeding schedule. My son has volume issue. Meaning he can only tolerate small amounts at a time. So we feed small amounts many times a day.</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">For him we give five 6-ounce servings of blended diet per day for a total of 30 oz. He gets feed a 7am, 11am, 2pm, 6pm and 9pm He also does eat and drink by mouth on his own so we mostly use blended diet to supplement what he eats on his own. But there some days he eats nothing and on those day he is on 100% blended foods. Every kid is different. Some will tollerate 8-10 oz three times day. While others need smaller amounts more often. We try to stick to the same meal/snack as someone without a g-tube would. </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Once total amount is determined, next the nutritional values need to be determined. How many calories does your child need? How much iron? How many grams of fiber, protein, carbs, etc. If your doctor is unable or unwilling to help with that here is a great <a href="http://fnic.nal.usda.gov/fnic/interactiveDRI/index.php" target="_blank">interactive calculator </a>based on your child's age, weight, height and activity level. The only thing it doesn't take into account it having EB. But our doctor has us add 500 calories to what is recommended based on his age, weight, height and activity level. For my son he needs between 2300-2500 calories a day to gain, grow and heal. I know that seems like a lot for a 4 1/2 year old bit it is working for him. </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">So your check list so far:</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">1) quantity </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">2) number of feedings</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">3) nutritional values needed per day</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Next you need a blender. If you are just starting out a basic kitchen blender will be just fine. We have a Vitamix that was donated to us from the company to use since my son has a g-tube. We contacted the company and provides medical documentation and they sent us a refurbished blender free of charge! But when we first started out, we used a regular blender with no issues. </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">Types of high quality blenders:</span></span><br />
<span style="font-size: small;"><span face="Verdana,sans-serif"><br /></span></span>
<span style="font-size: small;"><span face="Verdana,sans-serif"><a href="https://www.vitamix.com/Home" target="_blank">Vitamix </a></span></span><br />
<span style="font-size: small;"><span face="Verdana,sans-serif"><br /></span></span>
<span style="font-size: small;"><span face="Verdana,sans-serif"><a href="http://www.blendtec.com/" target="_blank">Blendtec </a></span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">Once you have determined:</span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">1) quantity </span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">2) number of feedings</span></span></div>
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<span style="font-size: small;"><span face="Verdana,sans-serif">3) nutritional values needed per day</span></span><br />
<span style="font-size: small;"><span face="Verdana,sans-serif">4) blender</span></span><br />
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<span style="font-size: small;"><span face="Verdana,sans-serif">You are now ready to move into preparing the foods!! </span></span><br />
<br />
<span style="font-size: small;"><span face="Verdana,sans-serif"><b><span style="font-size: x-small;"><i><span face="Verdana,sans-serif"><span style="color: black;">*Disclaimer: I am not a medical professional. This is not medical advice. i am just sharing what I have learned along the way with the hope it makes things easier for others. </span></span></i></span></b></span></span></div>
<span style="font-size: small;"><br /></span>
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</div><div style="text-align: center;" trbidi="on"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span></div>
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Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-62179015153892422132015-10-25T15:27:00.005-04:002021-01-14T14:24:55.537-05:00Just say no.....to Pedisure<div dir="ltr" style="text-align: left;" trbidi="on">
When a child with EB (or even without EB) get a G-tube it is very
common for the child to be feed a diet of only (or mostly) Pedisure (or
another commercial high calorie formula). It is a very convenient way to
provide nutrition with a g-tube but how nutritious is it really?<br />
<br />
Here are the ingredients in a bottle of Pedisure:<br />
<br />
<span style="font-size: small;"><span style="color: black; font-weight: bold;"><br /></span></span>
<span style="font-size: small;"><span style="color: black; font-weight: bold;">Water, Sugar, Corn Maltodextrin, Milk Protein Concentrate, High Oleic
Safflower Oil, Canola Oil, Soy Protein Isolate. Less than 0.5% of:
Short-Chain Fructooligosaccharides, Natural & Artificial Flavor,
Cellulose Gel, Potassium Chloride, Magnesium Phosphate, Potassium
Citrate, Calcium Phosphate, Tuna Oil, Calcium Carbonate, Potassium
Phosphate, Salt, Cellulose Gum, Choline Chloride, Ascorbic Acid, Soy
Lecithin, Monoglycerides, Potassium Hydroxide, m-Inositol, Carrageenan,
Taurine, Ferrous Sulfate, dl-Alpha-Tocopheryl Acetate, L-Carnitine, Zinc
Sulfate, Calcium Pantothenate, Niacinamide, Manganese Sulfate, Thiamine
Chloride Hydrochloride, Pyridoxine Hydrochloride, Riboflavin, Lutein,
Cupric Sulfate, Vitamin A Palmitate, Folic Acid, Chromium Chloride,
Biotin, Potassium Iodide, Sodium Selenate, Sodium Molybdate,
Phylloquinone, Vitamin D3, and Cyanocobalamin. <br />
<b>Contains milk and soy ingredients.</b></span></span><br />
<br />
<table cellpadding="0" cellspacing="0" style="width: 500px;"><tbody>
<tr><td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Calories</b>: 240</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Calories from Fat</b>: 80</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Fat, g</b>: 9</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">14 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;">
<b>Saturated Fat, g</b>: 1</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">5 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;">
<b>Trans Fat, g</b>: 0</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;">
<b>Polyunsaturated Fat, g</b>: 2</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;">
<b>Monounsaturated Fat, g</b>: 6</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Cholesterol, mg</b>: 5</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">2 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Sodium, mg</b>: 90</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">4 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Potassium, mg</b>: 350</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Carbohydrate, g</b>: 33</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">11 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;">
<b>Dietary Fiber, g</b>: 1</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">4 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;">
<b>Sugars, g</b>: 18</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Protein, g</b>: 7</td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">14 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td></tr>
</tbody></table>
<table cellpadding="0" cellspacing="0" style="width: 500px;"><tbody>
<tr><td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin A</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin C</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">40 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin D</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">40 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin E</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">20 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin K</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">20 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Thiamin</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">40 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Riboflavin</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">30 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Niacin</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin B6</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">30 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Folate</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">15 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Vitamin B12</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">25 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Biotin</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">15 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Pantothenic Acid</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">25 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Choline</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">15 </td></tr>
</tbody></table>
<table cellpadding="0" cellspacing="0" style="width: 500px;"><tbody>
<tr><td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Calcium</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">25 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Iron</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">15 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Phosphorus</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">20 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Iodine</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">15 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Magnesium</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Zinc</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Selenium</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Copper</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Manganese</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">20 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Chromium</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Molybdenum</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">10 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px;"><b>Chloride</b>: </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;">8 </td>
<td style="border-bottom: 1px dotted rgb(205, 205, 205); padding-bottom: 2px; text-align: center;"></td>
</tr>
<tr>
<td colspan="3"><sup>*</sup> Percent Daily Values (%DV) are based on a 2,000 Calorie diet. </td></tr>
</tbody></table>
<br />
<span style="font-size: small;"><span style="color: black; font-weight: bold;"><b> *************************************************</b></span></span><br />
<span style="font-size: small;"><span style="color: black; font-weight: bold;"><b>The following excerpts were taken from an article called: </b></span></span><br />
<h1>
<a href="http://www.healthbeginswithmom.com/why-pediasure-is-the-worst-thing-you-can-do-for-your-picky-eater/" target="_blank"><span style="font-size: small;">Why PediaSure Is The Worst Thing You Can Do For Your Picky Eater?</span></a></h1>
<span style="color: black; font-size: 1.4em; font-weight: bold;"><span style="font-size: x-small;"><b> </b></span></span> <br />
<i>"After water, the most prominent ingredient is sugar! How is that considered a nutritional supplement?! According to the nutrition label, one cup of serving has 240 calories –
80 calories are derived from fat, 25 from protein, and the remaining 135
are from carbohydrates. Now, here’s where it gets interesting – the
carb breakdown is just 1 gram of fiber, and 18 grams of sugar! That’s
the equivalent of <b>4.5 teaspoons of sugar per one bottle of PediaSure."</b></i><br />
<br />
<i>After sugar, it is followed by Corn Maltodextrin (GMO), which is used as
a thickener in soft drinks, salad dressings and soups. The fats in this
drink are derived from Safflower oil and Canola oil (which are also
GMOs). Can you imagine your child drinking genetically modified oil as a
source of their nutrients?</i><br />
<br />
<i>The proteins are derived from milk, soy, and peas as isolates and
concentrates. This means that the original, whole food is deconstructed
and highly processed, losing its beneficial nutrients."</i><br />
<br />
How on Earth can that be considered a HEALTHY, nutritional meal for a child with constant open wounds?!?!? Most kids with EB get 4 cans of that over a 24 hour period. 18 grams of sugar per can, times 4 cans, equals 72 grams of SUGAR A DAY!!!!!! That is about the equivalent of two 12-oz cans of Coke PER DAY. And that is pretty much the entire daily diet for many kids with EB who have G-tubes.<br />
<br />
Sugar is one of the main causes of inflammation within the body. And EB wounds are often causes by chronic inflammation. So only eating or being feed nutrition that is is known to cause chronic inflammation just creates a vicious cycle of non-healing wounds and chronic inflammation. I truly believe that these commercial, high sugar, inflammatory causing "nutritional" beverages are contributing to the rapid decline of so many kids with EB.<br />
<br />
There is however, an alternative that is slowly catching on within the EB community!! And that alternative is something called The Blended Diet. It is what is sounds like, normal food that normal people eat but blended into a liquid or puree state that allows it to be pushed thru a g-tube using a syringe. We have been going the blended diet route for almost 3 years now and the results have been overwhelming positive. My son is a healthy, happy, very active preschooler who has ZERO chronic wounds, does not have any of the chronic complications that arise in EB such as anemia, constipation, vitamin or mineral deficiencies.<br />
<br />
This week I plan to create a multi-part series about all the things one needs to know before start down the blended diet road. It is not as complicated as it sounds either. It can be time consuming, but once you get the basics down, it becomes an easy thing to incorporate into your daily routine! <br />
<br />
<br />
<span style="color: black; font-size: 1.4em;"><span style="font-size: x-small;"><b><span style="font-size: x-small;"><i><span face="Verdana,sans-serif"><span style="color: black;">*Disclaimer: I am not a medical professional. This is not medical advice. i am just sharing what I have learned along the way with the hope it makes things easier for others. </span></span></i></span></b> </span></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="color: black; font-size: 1.4em;"><span style="font-size: x-small;"> </span></span></div><div style="text-align: center;" trbidi="on"><span style="color: black; font-size: 1.4em;"><span style="font-size: x-small;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span> </span></span><span style="color: black; font-size: 1.4em; font-weight: bold;"> </span></div>
Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-59996978192097417312015-05-05T11:28:00.036-04:002021-01-14T14:25:21.848-05:00Testing for EB<div style="text-align: center;"><span style="font-family: georgia;"><span style="font-size: large;">Why Should Genetic Testing be done?</span></span><p style="text-align: center;"><span style="font-family: georgia;"><span style="font-size: large;"> </span></span></p><p style="text-align: left;"><span style="font-family: georgia;">There are actually many reasons to have genetic testing done. Here are just a few<br /></span></p><p style="text-align: left;"><span style="font-family: georgia;">~ much more accurate than a biopsy</span></p><p style="text-align: left;"><span style="font-family: georgia;">~ will not only give you the type of EB, but subtype too</span></p><p style="text-align: left;"><span style="font-family: georgia;">~aids in family planning; not only for parents, but for the child as well</span></p><p style="text-align: left;"><span style="font-family: georgia;">~ allows for other family members to be testing to see if they are carriers of the same mutation(s)</span></p><p style="text-align: left;"><span style="font-family: georgia;">~ offers insight as to the prognosis of your form of EB</span></p><p style="text-align: left;"><span style="font-family: georgia;">~prepares you for which complications you need to be on the look out for</span></p><p style="text-align: left;"><span style="font-family: georgia;">~allows
you to participate in clinical trials and potential be one of the first
to receive the cure when it is found. (otherwise you'd have to have
genetic testing done prior to participating any clinical trial/cure)</span></p><p style="text-align: left;"><span style="font-family: georgia;"><br /></span></p><p style="text-align: left;"><span style="font-family: georgia;">Once you have received your results, here is a list of question to ask, if the doctor doesn't already answer them.</span></p><p style="text-align: left;"><span style="font-family: georgia;">1) How many mutations were found?</span></p><p style="text-align: left;"><span style="font-family: georgia;">2) Which gene(s) were they found on?</span></p><p style="text-align: left;"><span style="font-family: georgia;">3) Which form(s) of EB are these mutations associated with? </span></p><p style="text-align: left;"><span style="font-family: georgia;">4) Have the mutations ever been reported before in others with EB?</span></p><p style="text-align: left;"><span style="font-family: georgia;">5) Are the mutations homozygous or heterozygous? </span></p><p style="text-align: left;"><span style="font-family: georgia;">6) What is the inheritance pattern for the mutation(s)?</span></p><p style="text-align: left;"><span style="font-family: georgia;">7) Should, we have parents be, tested? Should our other children be tested as well?</span></p></div><div style="text-align: center;"><br /></div><div style="text-align: left;" trbidi="on">
<span style="font-family: georgia;"><b><span class="mainpagetitle"> </span></b></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><b><span class="mainpagetitle"> </span></b></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><b>Genetic Testing </b><br /></span>
<span style="font-family: georgia;"><br />
Molecular genetic studies (DNA analysis) is a must more accurate way to diagnosis EB. Molecular studies are done to
identify the specific genetic mutation and to determine the mode of
inheritance (recessive vs. dominant) There is a new genetic test that tests for ALL known EB mutations in one sample: It is called the <a href="https://www.genedx.com/test-catalog/available-tests/epidermolysis-bullosa-eb-xomedxslice/"><span style="font-size: small;"><span>Epidermolysis Bullosa (EB) XomeDx<sup>®</sup><i>Slice </i></span></span></a></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><span style="font-size: small;"><span><i> </i></span></span></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><span style="font-size: small;"><span><i> </i></span></span>
<br /></span>
<span style="font-family: georgia;">Below are two labs in the US that can perform genetic test for EB.<br /></span>
<span style="font-family: georgia;"><br />
1) GeneDx<br />207 Perry Parkway<br />Gaithersburg, MD 20877<br />301-519-2100<br /><a href="http://www.genedx.com/" target="_blank">www.genedx.com</a><a href="http://www.genedx.com/" target="_blank"> </a><br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><a href="http://www.cincinnatichildrens.org/service/d/diagnostic-labs/molecular-genetics/default/" target="_blank">2) The Molecular Genetics Laboratory at Cincinnati Children’s Hospital</a></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> </span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> </span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> </span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> </span></div><div style="text-align: left;" trbidi="on"><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><b><span class="mainpagetitle">Skin Biopsy for EB</span></b><br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><span class="mainpagetitle">Though
not the go-to in EB diagnostics anymore, here is the information for those that genetic testing is not a option for.</span></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><span class="mainpagetitle"> </span></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><span class="mainpagetitle">Samples of the skin or a
blister may taken by a trained doctor to determine the type of EB one
has. </span><span class="mainpagetitle">The skin samples must be processed for specific studies,
immunofluorescence antigen (IFA) mapping and transmission electron
microscopy (EM). </span></span></div><span style="font-family: georgia;"><span class="mainpagetitle"> </span><br />
Immunofluorescence antigen mapping is performed to identify exactly
where the blister has occurred and which proteins are involved (absent
or diminished in amount). This is a specialized study that should be
done by a pathologist in a laboratory that specializes in this
procedure.<br /></span>
<span style="font-family: georgia;"><br />
Only two labs in the US can process EB biopsies:<br /></span>
<span style="font-family: georgia;"><br />
1) Beutner Labs, Inc.<br />3580 Harlem Road<br />Buffalo, NY 14215<br />1-800-288-0549<br /><a href="http://www.beutnerlabs.com/contact/" target="_blank">http://www.beutnerlabs.com/contact/</a><br /></span>
<span style="font-family: georgia;"><br />
2) Stanford Dermatopathology Service<br />Department of Pathology - H2110<br />Stanford Medical Center<br />300 Pasteur Drive<br />Stanford, CA 94305<br />650-723-6736</span><span style="font-family: georgia;"> <br /></span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> </span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> ***********************************************************</span></div><div style="text-align: left;" trbidi="on"><span style="font-family: georgia;"> </span></div><br /><div style="text-align: left;" trbidi="on"></div><div style="text-align: left;" trbidi="on"><br /></div><div style="text-align: center;" trbidi="on"><span style="font-family: georgia;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span></span>
<span style="font-family: georgia;"><span class="mainpagetitle"> </span></span></div><span style="font-family: georgia;">
</span>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-126444648808297082015-01-22T10:12:00.001-05:002021-01-14T14:25:33.887-05:00blended diets<div dir="ltr" style="text-align: left;" trbidi="on">
Instead of the pre-made commercial formula, we choose to give Joey a blended diet. A blended diet is when you take real food and blended up to the consistency of a smoothie. We then feed like a set volume at every meal. <br />
<br />
Here is a basic guide I came up with that would be easy for someone new to the blended diet world to follow. <br />
<br />
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<span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">The main ingredient we use is Kefir
(it is a yogurt drink)</span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<i>32oz </i></span><a href="http://www.lifeway.net/Products/Kefir.aspx" target="_blank"><i><span face=""Arial","sans-serif"" style="color: blue; font-size: 12pt; mso-fareast-font-family: "Times New Roman";">Kefir</span></i></a><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"> (560 calories 44g protein)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">Next I use one 16oz serving of a
fruit and one 16oz serving of a veggie. I mix and match each time
depending on what is available at the store and/or on sale. Here are the
fruits and veggies we have tried. </span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">16oz servings:</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<i><br />
</i></span><b><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">FRUITS </span></i></b><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">3 large apples diced (288 calories
16.2g fiber 1.6g protein)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">2 mangos diced (214 calories 6g
fiber 3% iron)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">3 large pears diced (288 calories
15g fiber 6% iron)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">3 large peaches diced (114 calories
5.5g fiber 3% iron)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">3 large bananas chopped (315
calories 9g fiber 3g protein)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">1 pint of Blue Berries (194
calories, 8.2g Fiber 2.52g protein, 5% Iron) </span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<i><br />
</i></span><b><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">VEGGIES </span></i></b><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">1 large sweet potato diced (480
calories 6g fiber 3g protein)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">1 bag baby carrots chopped (159
calories 6g fiber 3g protein)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">2 bunches kale (68 calories 3.9g
fiber 6.6g protein)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">2 Avocados (454 calories 12.9 fiber
4.2g protei</span></i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">n)</span></div>
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<span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">Green Beans, Peas, Mixed Veggies,
Squash, Pumpkin (canned or fresh) are other options we have used but I don’t
have the nutritional value for.</span></div>
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<br /></div>
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<span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">Try to stay away from fruits and
veggies that are acidic as they can irritate those with acid reflux. </span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<br />
<br />
<br />
</span><b><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">GRAINS-</span></b><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"> I recently started experimenting with using a grain...this
is what I have tried thus far.<b> </b>I don't use it every time though </span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><b><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span></b><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">1 1/3 cups cooked minute rice multi
grain medley (brown rice, red rice, wild rice, quinoa)<br />
320 calories 4g <u>fiber</u> 8g protein 16% zinc 8% iron</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<br />
<br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">I blend the fruits, veggies and
Kefir with 8oz prune juice (180 calories 3g fiber 4% iron 4% zinc)</span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
Once this is all blended it makes about 72 oz of blended food. I then
divide it into five 16oz glass mason jars. I then add the following to EACH mason
jar: </span></div>
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<span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">3 tablespoon Happy Bellies Organic
Baby Oatmeal (60 calories 2g fiber 10% zinc 80% iron.)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<i><br />
</i></span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">1 tablespoon olive oil or coconut
oil (120 calories)</span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<i><br />
</i></span><i><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">1 tray (1.5oz) of Benecalorie (330
calories.) </span></i><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">Each container comes out to around
1300 calories each. He gets 7-8oz servings 4 times a day depending on how
much he each on his own by mouth. I make his blended food about 3 times a
week. </span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
In addition to the blended diet he also gets 7oz of 30cal/oz Elecare Jr.
Formula and 120 cc of free water per day.</span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
<br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";">The doctor wants him between
1800-2000 calories a day when possible :) He needs those calories for
wound healing, energy (which he had a TON of) and to gain/grow.
</span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
</span><span face=""Arial","sans-serif"" style="font-size: 12pt; mso-fareast-font-family: "Times New Roman";"><br />
There are many blended diet recipes out there and this is just an example of
what we use! Yes this is a lot of work, but I think Joey is healthier
because of it!</span><span style="font-family: "Times New Roman","serif"; font-size: 12pt; mso-fareast-font-family: "Times New Roman";"> </span></div>
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<br /></div><div class="MsoNormal" style="text-align: center;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span>
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Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-67355573072609128972014-10-24T21:56:00.002-04:002021-01-14T14:26:05.510-05:00EB Medical Directory<div dir="ltr" style="text-align: left;" trbidi="on">
<div style="text-align: center;">
<span style="font-family: georgia;"><span style="font-size: large;"><b>Important EB Related Phone Numbers </b></span></span></div>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><br /> Debra's <a href="https://www.debra.org/get-help/eb-nurse-educator-program" target="_blank">EB Nurse</a> Educator Program</span></div><div dir="ltr" style="text-align: left;" trbidi="on"><p><span style="font-family: georgia;"><b>Phone (toll-free):</b> 866-DEBRA-RN (866-332-7276)<br /></span>
<span style="font-family: georgia;"><b>Email: </b><a href="mailto:nurse@debra.org">nurse@debra.org</a></span></p><span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;">Wound Care Supplier <a href="http://nationalrehab.com/index.html" target="_blank">AdaptHealth </a>(Formally known as National Rehab and McKesson)<br />
855-5EB-LINE<br />
8a-5p (EST)<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>Cincinnati Children’s Hospital Medical Center, </b>Cincinnati, OH<br />
Contact Donna Engells<br />
Clinic Coordinator (513) 636-2009<br />
http://www.cincinnatichildrens.org/health/e/epidermolysis-bullosa/ <br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>Stanford School of Medicine</b>, Palo Alto, CA<br />
Contact Loraine Spaulding<br />
Clinic Coordinator (650) 804-4820<br />
http://dermatology.stanford.edu/gsdc/eb_clinic/ <br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>The Hospital for Sick Children</b><br />
Toronto, Canada<br />
(416) 813-1500- ask for the EB Clinic<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>University of Massachusetts</b><br /></span>
<span style="font-family: georgia;"><b> </b>UMMHC, Hahnemann Campus<br />
281 Lincoln Street<br />
Worcester, MA 01605<br />
Phone: 508-334-5979 - ask for EB Clinic<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>Herbert Irving Center for Dermatology and Skin Cancer</b><br />
Herbert Irving Pavilion, 12th Floor<br />
161 Fort Washington Avenue<b>, </b>New York, NY<b> </b><br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>Columbia University Dermatology Associates</b><br />
16 East 60th Street, Suite 300<br />
New York, NY 10022<br /></span>
<span style="font-family: georgia;"><b>To make an appointment at either location call</b><b> 212-305-5293</b>.<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>Phoenix Children’s Hospital</b><br />
1919 E. Thomas Road, Phoenix, AZ 85016<br />
(602) 933-2053<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>The Children’s Hospital Aurora</b><br />
13123 East 16th Avenue<br />
Aurora, CO 80045<br />
720-777-8445<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>Sydney Children's Hospital</b><br />
Sydney, Australia<br />
Contact # 9382-1470 (not sure what the area or country code is)<br /></span>
<span style="font-family: georgia;"><br /></span>
<span style="font-family: georgia;"><b>There are other clinic locations. To find one near you contact <a href="http://debra.org/" rel="nofollow" target="_blank">DebRA.org</a></b></span></div><div dir="ltr" style="text-align: left;" trbidi="on"><span style="font-family: georgia;"><b> </b></span></div><div style="text-align: center;" trbidi="on"><span style="font-family: georgia;"><b><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span> <br /></b></span></div><span style="font-family: georgia;">
</span>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-76494203801105374632014-10-20T12:51:00.002-04:002021-01-14T14:26:45.861-05:00Heal EB - Cause The Wave<iframe allowfullscreen="" frameborder="0" height="270" src="https://www.youtube.com/embed/S0XJGiNOnHI" width="480"></iframe>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-11814709037996036722014-10-10T10:11:00.006-04:002021-01-14T14:26:57.370-05:00Pain Management Recomendations in EB<div dir="ltr" style="text-align: left;" trbidi="on">
<div style="text-align: center;">
<br /></div>
<div style="text-align: center;">
<br /></div>
<div style="text-align: center;">
<span style="font-size: large;">A new article was published yesterday on pain management in EB:</span></div>
<div style="text-align: center;">
<br /></div>
<h1 style="text-align: center;">
<a href="http://www.biomedcentral.com/1741-7015/12/178#B16" target="_blank">Pain care for patients with epidermolysis bullosa: best care practice guidelines</a></h1><h1 style="text-align: center;"> </h1><h1 style="text-align: center;"> </h1><h1 style="text-align: center;"> </h1><h1 style="text-align: center;"> </h1><h1 style="text-align: center;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span> <br /></h1>
</div>
Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-4498081899221701722014-10-01T23:37:00.003-04:002021-01-14T14:27:24.323-05:00Cause The Wave: Eddie Vedder and the EB Research Partnership<p></p><p></p><p></p><p></p><p><iframe allowfullscreen="" frameborder="0" height="270" src="https://www.youtube.com/embed/hXN4GpisLMU" width="480"></iframe></p>Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0tag:blogger.com,1999:blog-7961655801018531468.post-65767434130125210572014-10-01T14:32:00.002-04:002021-01-14T14:27:34.229-05:00JGSF and Heal EB annouce Merger<div dir="ltr" style="text-align: left;" trbidi="on">
<h1 style="text-align: center;">
<span face="Verdana,sans-serif"><span style="font-size: large;"><a href="http://www.prnewswire.com/news-releases/jackson-gabriel-silver-foundation-and-heal-eb-announce-merger-277734321.html" target="_blank">Jackson Gabriel Silver Foundation and Heal EB Announce Merger</a></span></span></h1>
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<a href="http://ebresearch.org/" target="_blank"><img alt="http://ebresearch.org/" border="0" height="137" src="http://1.bp.blogspot.com/-B3alrC1dwEA/VCxIPPKcl-I/AAAAAAAACxk/sf6LX-7XaLU/s1600/eb_weblogo1.jpg" width="400" /></a></div>
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</h1><h1> </h1><h1> </h1><h1 style="text-align: center;"><span style="font-family: georgia;"><span style="font-size: medium;"><span>©Garrett's House 2021</span></span></span> <br /></h1>
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Sara Denslawhttp://www.blogger.com/profile/09625588629095084632noreply@blogger.com0