Epidermolysis bullosa

Alternative Names

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

Definition of Epidermolysis bullosa

Epidermolysis bullosa is a group of inherited disorders in which skin blisters develop in response to minor injury.

Causes, incidence, and risk factors

There are four main types of epidermolysis bullosa:

Symptoms

Symptoms depend on the type of epidermolysis bullosa, but can include:

Signs and tests

Your doctor may suspect epidermolysis bullosa based on the appearance of the skin.

Treatment

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

Expectations (prognosis)

What happens depends on how severe the illness is. Mild forms of epidermolysis bullosa improve with age. Scarring from several types of epidermolysis bullosa can restrict mobility and impair daily activities. Lethal forms of epidermolysis bullosa have a very high mortality rate. (See Complications section.)

Review

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. – 10/31/2008

Epidermolysis bullosa, dominant dystrophic
Epidermolysis bullosa, dystrophic

ADAM Medical Encyclopedia

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