EB can vary from minor to fatal. The minor forms causes blistering of the skin. The fatal forms affect other organs. Most types of this condition start at birth or soon after. It can be hard to identify the exact type of EB a person has, although specific genetic markers are now available for most.
Family history is a risk factor. The risk is higher if a parent has this condition.
Another rare type of EB is called epidermolysis bullosa acquisita. This form develops after birth. It is an autoimmune disorder, which means the body attacks itself.
Symptoms
Depending on the form of EB, symptoms can include:
Skin tests may be used to identify the form of EB.
Other tests that may be done include:
Blood test for anemia
Culture to check for bacterial infection if wounds are healing poorly
Imaging studies to look at other organs
Growth rate will be checked often for a baby who has or may have EB.
Treatment
The goal of treatment is to prevent blisters from forming and avoiding complications. Other treatment will depend on how bad the condition is.
HOME CARE
Follow these guidelines at home:
Take good care of your skin to prevent infections.
Follow your provider's advice if blistered areas become crusted or raw. You might need regular whirlpool therapy and to apply antibiotic ointments to wound-like areas. Your provider will let you know if you need a bandage or dressing, and if so, what type to use.
You may need to use oral steroid medicines for short periods of time if you have swallowing problems. You may also need to take medicine if you get a candida (yeast) infection in the mouth or throat.
Take good care of your oral health and get regular dental check-ups. It is best to see a dentist who has experience treating people with EB.
Eat a healthy diet. When you have a lot of skin injury, you may need extra calories and protein to help your skin heal. Choose soft foods and avoid nuts, chips, and other crunchy foods if you have sores in your mouth. A nutritionist can help you with your diet.
Do exercises a physical therapist shows you to help keep your joints and muscles mobile.
If your infant has any blistering shortly after birth, contact your provider. If you have a family history of EB and plan to have children, you may want to have genetic counseling.
Prevention
Genetic counseling is recommended for prospective parents who have a family history of any form of epidermolysis bullosa.
During pregnancy, a test called chorionic villus sampling may be used to test the baby. For couples at high risk of having a child with EB, the test can be done as early as week 8 to 10 of pregnancy. Talk to your provider.
To prevent skin damage and blistering, wear padding around injury-prone areas such as the elbows, knees, ankles, and buttocks. Avoid contact sports.
If you have EB acquisita and are on steroids for longer than 1 month, you may need calcium and vitamin D supplements. These supplements may help prevent osteoporosis (thinning bones).
Review Date:
11/30/2022
Reviewed By:
Ramin Fathi, MD, FAAD, Director, Phoenix Surgical Dermatology Group, Phoenix, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.