Junctional epidermolysis bullosa; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Weber-Cockayne syndrome

The goal of treatment is to prevent blisters from forming and complications. How much treatment is needed depends on how severe the disease is. Recommendations often include avoiding skin damage (trauma) and hot environments.

• To prevent infection take very good care of the skin, especially if any blistered areas become crusted or exposed (raw). Follow your health care provider's instructions closely. You might need regular whirlpool therapy and to apply antibiotic ointments to wound-like areas. Your health care provider will let you know if you need a bandage or dressing, and if so, what type to use.
• For swallowing difficulties, your may need to use oral steroids for short periods of time. Long-term steroids for epidermolysis bullosa is generally not recommended. If you also have candida in the mouth or esophagus, you will also need to take medication for that infection.
• Good dental hygiene is very important, including regular dental visits. It is best to see a dentist who has experience treating people with epidermolysis bullosa.
• Proper nutrition is also important. When you have a lot of skin injury, you may need extra calories and protein to help you recover. Work closely with a nutritionist. If you have blisters or complications in the mouth or esophagus, avoid eating hard or brittle foods such as pretzels, nuts, and chips. Eating soft foods can help prevent making the sores worse.
• Working with a physical therapist can help you keep the full range of motion in the joints and minimize contractures.

Skin grafting for denuded or ulcerated areas of the skin may be necessary. Other surgical procedures for complications of epidermolysis bullosa might be recommended. Such surgeries include:

• Dilation of the esophagus if there is a narrowing (stricture)
• Repair of hand deformities
• Removal of of any squamous cell carcinoma that develops

Other treatments under investigation for epidermolysis bullosa include protein and gene therapy.

Epidermolysis bullosa acquisita may be treated with oral steroids and medication that suppresses the immune system. These may, however, increase the risk of infection or damage the liver or kidneys. Studies using interferon are also under way.

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Epidermolysis bullosa, dystrophic

Epidermolysis bullosa, dominant dystrophic

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Review Date: 10/10/2010
Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine. Also reviewed by Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)