Determing Severity in Epidermolysis Bullosa

An article published in January 2025 shared a more accurate way of identifying severity in EB. 

Mild EB:

Splice B/Missense

Missense/Missense

Moderate EB:

PTC/Splice B

Splice A/Splice B

Splice A/Missense

Splice B/Splice B

Severe EB:

PTC/PTC

PTC/Splice A

Splice A/Splice A

PTC= premature termination codon

PTC AKA Nonsense 

Splice A: a downstream PTC mutation 

Spice B: an in-frame Exon Skipping

Infections in EB: How to prevent them and how to treat them

While skin infections in EB are common, there are several measures one can take to prevent them. The following advice was given to me by an infectious disease doctor.

Prevention

1. ALL wounds need to be bandaged. Open wounds can easily become contaminated with bacteria, causing an infection to start. Keeping wounds covered with bandages as they heal not only prevents an infection from occurring, but also protects the wounds from further damage while they heal.

2. Bandages need to be changed daily, and the skin needs to be cleaned daily. Some bandages need to be changed multiple times a day if there is heavy drainage.  The longer infection drainage sits on a wound, the more infected it becomes and the longer it takes to heal.

3.  ONLY use bleach when you have an ACTIVE staph or strep infection, and no more than 2 times a week. Since it is cytotoxic, it will kill both good and bad bacteria.  Chlorhexidine, also known as Hibiclens, Peridex, and Periogard, is also cytotoxic. 

4.  Take showers, not baths.  Sitting in a tub of water with open wounds allows bacteria to spread to other open wounds.  A shower doesn't have to be like it sounds.  It can be sitting in an infant bathtub, making up a bowl of warm water and soap (or whatever you use in the bath), and gently pouring it over the body to clean it.  It could also be letting a toddler sit in the bathtub with just enough water to play in, and using a bowl or pitcher of warm, soapy water to clean them with.  However you do it, clean the bathtub before and after bath/shower time.

5. Wash the clothing, bedding, towels, and blankets of those with EB separately from everyone else in the household to prevent the spread of bacteria.  Changing bedding daily when there is an active infection, and using clean towels every time you bathe/shower can also help reduce the spread of the bacteria. 

6.  Frequently clean your child's toys and other objects in the house that they touch a lot- the TV remote, computer mouse/keyboard, door knobs, light switches, the toilet, fridge handle, etc.

7. Change clothing after coming home from school, work, and medical appointments.

Signs of an infection

• red or swollen wound, sometimes it will feel hot
• foul odor
• excessive drainage (more than usual)
• excessive pain and/or itching (more than usual)
• a fever

(Greenish-blue drainage can indicate a Pseudomonas infection. Cloudy, pea-green, or yellowish drainage can indicate a strep or staph infection.)

Only a wound culture performed by a doctor can tell you what type of bacteria is causing the infection. 

The typical treatment of an infection

Treatment depends on the person's age, previous history of infections, and the recommendation of their medical team.

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. Overuse of it can cause your body to become resistant to it and it will no longer work when you really need it to. The same thing applies to oral antibiotics. Use those only when necessary so your body doesn't become resistant to them either.  In addition to topical and oral antibiotics, there are other home remedies that you can use to help eliminate the bacteria. 

For Pseudomonas Infection, you will want to use an acetic solution, and the most common thing to use is  White Vinegar. 

The acetic acid formula is: Mix 1 ounce of 5% vinegar in 19 ounces of water.

Vinegar baths or compresses can be used at any age. Be sure you rinse well with clear, clean water after a Vinegar bath; otherwise, excess itching and irritation may occur. 

For a Staph or Strep-related infection (including MRSA), you can take a bleach bath or apply bleach compresses to the infection area to help kill the bacteria no more than 3 times a week. Bleach is cytotoxic (meaning it kills both bad and good bacteria. When you kill off the good bacteria, things like pseudomonas and yeast start to take over) 

Bleach Bath Formula for Adults and Children OVER the age of 1:

Mix 1 teaspoon of bleach in 1 gallon of water.

For Children and Infants UNDER the age of 1, 

Mix 1/2 teaspoon of bleach or less into 1 gallon of water.

No matter what age, rinse well with clear, clean water after a bleach bath, otherwise, excessive itching and irritation may occur.

NEVER mix Vinegar and Bleach together in the same bath. A chemical reaction can occur and produce toxic fumes, 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 is also vital to use the correct ratio of water to bleach, or vinegar to water.  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

How WE treat infections.

1- Cover all open wounds

2- Clean the wounds and change bandages at least once a day, sometimes more for heavily draining wounds.  

3- We start with topical antibiotics and/or antimicrobial dressings before we use oral antibiotics, UNLESS we have multiple infected wounds and/or a fever is present.  

Some examples of antimicrobial dressings: Xeroform (NOT safe for use on Infants) Mepilex AG, Polymen AG or Biatain AG (also NOT safe for use on infants or young children) Hydrofera Blue Ready Foam,  and dressings impregnated with medical grade honey,   There are also topical antimicrobial agents: silver gel (NOT safe for use on infants or young children) MediHoney, essential oils that have been safely diluted for the age of the person.

Since we switched from baths to showers and followed the above steps, we have seen a significant reduction in the occurrence of infections. 

©Garrett's House 2026

Updated Recommended Blood Work for those with EB

**New Release January 2024**

Recommend Blood Work for All Forms of EB starting at age 1 OR for those who have never had blood work done before

TSH

T4 Free

Vit D3

CRP

Sed Rate

CMP (comprehensive metabolic which includes liver and kidney functions)

CBC w/ Differential 

Vit C

B12

Zinc

Other Yearly Tests that are Recommended for all forms of EB

EKG 

Echocardiogram

Dexa Scan

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:

CRP

Sed Rate

CMP (comprehensive metabolic which includes liver and kidney functions)

CBC w/ Differential 

Vit C

Zinc

Vit D3

Additional recommended test for those with RDEB, JEB or severe EBS and/or have a history of anemia, liver and/or renal issues

Ammonia

Full Iron Panel

Copper

Expanded Hepatic Panel

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. 

• Vitamin A

• B Vitamins

• Thiamin – Vitamin B1

• Riboflavin – Vitamin B2

• Niacin – Vitamin B3

• Pantothenic Acid – Vitamin B5

• Vitamin B6

• Biotin – Vitamin B7

• Folate (Folic Acid)

• Vitamin B12

• Vitamin C

• Vitamin D

• Vitamin E

• Vitamin K

• Choline

• Calcium

• Chloride

• Chromium

• Copper

• Fluoride

• Iodine

• Iron

• Magnesium

• Manganese

• Molybdenum

• Nickel

• Phosphorus

• Potassium

• Selenium

• Sodium

• Zinc

 

Heavy Metals: Arsenic, Cadmium, Cobalt, Lead, Mercury 

©Garrett's House 2026

Our arsenal of ointments in wound care

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"

Between my 4+ decades living with EB and trying different things with the kids, we have tried sooooo many different ointments, lotions, oils, etc...

Below are the ointments we use the most.  I'll also explain what type of wound we use each on. 

** DISCLAIMER**

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.

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 brand we have used before.

In place of Aquaphor, I like Burt's Bee Multipurpose Ointment.  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.

For use around G-tubes sites and the wounds in the diaper area we use Medline Remedy 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.

For intact, but dry and itchy skin, we like the following three creams.  Aveeno Dry Skin Cream and Eucerin Skin Calming Cream and Sween Cream. 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. 

For smaller, non deep wounds, irritated skin I recently discovered this ointment, Cetaphil Soothing Gel-Cream.  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. 

For deep wounds that need moisture to heal, I use hydrogel, any brand.  It is long last and is safe to use on large areas or open or deep wounds.

For the thick callouses we have on our feet (common in generalized severe EBS) we found that Kerasal Foot ointment is helpful in reducing the roughness of the callouses.  I wouldn't use anywhere near an open wound though

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.

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. 

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:

4oz vick's vapor rub

1/2 tube of polysporin antibiotic ointment  

1/2 tube of hydrocortisone cream

1/2 tube of Hemorrhoid Treatment Cream (for pain relief)

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.  

 

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.