Epidermolysis Bullosa

Epidermolysis bullosa (EB) is an inherited connective tissue disease causing blisters in the skin and mucosal membranes, with an incidence of 1/50,000. Its severity ranges from mild to lethal. It is caused by a mutation in the integrin α6β4 cell adhesion molecule on either the alpha or beta subunit.

As a result, the skin is extremely fragile. Minor mechanical friction or trauma will separate the layers of the skin and form blisters. People with this condition have an increased risk of cancers of the skin, and many will eventually be diagnosed with it as a complication of the chronic damage done to the skin.

Phoenix Children’s Hospital flickr (link)

Epidermolysis bullosa refers to a group of inherited disorders that involve the formation of blisters following trivial trauma

 A patient has surgery to release tendons in severe EB (link)

DEBRA of America -Dystrophic Epidermolysis Research Association (link)

EB info world is a blog to support families.  Includes photographs of different types of EB. (link)

Recessive dystrophic epidermolysis bullosa (DEB)        Courtesy of Daniel Eisen, MD

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