Epidermolysis Bullosa - Signs, Care and Treatments
Epidermolysis bullosa (EB) is a group of rare, inherited conditions that make the skin so fragile it blisters or tears from minor rubbing or bumps. It's sometimes called "butterfly skin." Most people with EB are diagnosed in infancy or childhood, but the condition affects whole families, including parents and grandparents who help with daily care. This guide explains the signs of EB, how it's diagnosed, how it's cared for day to day, and the FDA-approved treatments now available, including newer gene therapies for dystrophic EB.
The Main Types of Epidermolysis Bullosa
| Type | Where blisters form | What to know |
|---|---|---|
| EB simplex | Top layer of skin | The most common type; often milder, with blisters on the hands and feet. |
| Junctional EB (JEB) | Between skin layers | Can be severe, with blisters beginning in infancy. |
| Dystrophic EB (DEB) | Deeper layer of skin | Caused by changes in the COL7A1 gene. The recessive form (RDEB) is often severe, with scarring and complications. |
| Kindler EB | Multiple layers | Very rare; may include sun sensitivity. |
Signs and Symptoms of EB
- Blisters or skin tears from light friction, bumps, or even heat
- Blisters inside the mouth or throat, which can make eating painful
- Slow-healing wounds and scarring
- Thickened skin on the palms and soles, or nail changes and loss
- In some types, fused fingers or toes, narrowing of the esophagus, or eye irritation
Signs usually appear at birth or in early childhood. Any newborn or child with unexplained, recurring blisters should be seen by a doctor.
How EB Is Diagnosed
- A skin biopsy: a small sample examined with special staining (immunofluorescence mapping) or electron microscopy shows where in the skin the blisters form.
- Genetic testing: identifies the specific gene change, which confirms the type of EB, guides treatment, and is often required by insurers for the newest therapies.
- Family history and genetic counseling: helps families understand inheritance and plan for future pregnancies.
Day-to-Day EB Care
- Wound care: gentle cleaning, draining blisters as directed by your care team, and non-stick dressings.
- Preventing new blisters: soft, loose clothing, padding, and careful handling.
- Pain and itch management: both are common and treatable.
- Infection prevention: watching for redness, warmth, or drainage, and treating infections promptly.
- Nutrition: mouth and throat blisters can make eating hard, so many people need nutritional support.
- Regular skin checks: people with severe dystrophic EB have a higher risk of a type of skin cancer, so routine checks are important.
A multidisciplinary EB center, with dermatologists, wound care nurses, nutritionists, and other specialists, offers the most complete care. For more on the dystrophic form, see our guide to dystrophic EB signs and care.
FDA-Approved Treatments for Epidermolysis Bullosa
For years, EB care meant wound care alone. Since 2023, the FDA has approved several treatments:
| Treatment | Type of EB | How it works |
|---|---|---|
| Vyjuvek (beremagene geperpavec) | Dystrophic EB with COL7A1 gene changes | The first FDA-approved treatment for DEB (2023), and a topical gene therapy gel applied to wounds, usually once a week. In 2025, approval was extended to newborns. |
| Filsuvez (birch triterpenes) | Junctional and dystrophic EB, ages 6 months and older | A topical gel made from birch bark extract that helps partial-thickness wounds close. It is not a gene therapy and can be used at home. |
| Zevaskyn (prademagene zamikeracel) | Recessive dystrophic EB, children and adults | Approved in April 2025. Sheets made from the patient's own gene-corrected skin cells are applied to larger wounds in a single procedure at specialized treatment centers. |
What families should know:
- These treatments are prescribed by specialists, and insurers usually require prior authorization, often including genetic test results confirming the type of EB.
- Treatments are generally not used together on the same wounds; your care team will choose the best option.
- Wound care, pain control, nutrition, and infection prevention remain essential alongside any new therapy.
- Clinical trials continue to test new approaches; your EB center can tell you if you may be eligible.
Blistering That Starts in Adulthood
Inherited EB almost always begins in childhood. If an older adult develops new, unexplained blisters, a doctor should look for other causes. These include autoimmune blistering conditions, such as epidermolysis bullosa acquisita (a rare, non-inherited condition) and bullous pemphigoid, which is more common in older adults, as well as reactions to medications. A dermatologist can diagnose these with a skin biopsy. Another rare condition that affects the skin is dermatomyositis.
Support for Families and Caregivers
- Patient organizations such as DEBRA of America and EB Research Partnership offer education, care resources, and research updates.
- Specialized EB centers coordinate care and can connect families with clinical trials.
- Caregiver support: daily wound care is demanding, and help from family, home health aides, or respite care makes a difference. For tips, see our guide for night shift caregivers.
The Bottom Line
Epidermolysis bullosa is rare and lifelong, but care has changed significantly in recent years. Early diagnosis with a skin biopsy and genetic testing, expert wound care, and FDA-approved treatments such as Vyjuvek, Filsuvez, and Zevaskyn give families more options than ever. Work with an EB specialist to find the right plan.
This article is general information, not medical advice. Talk with a dermatologist or EB specialist about diagnosis and treatment.