Complete Medical Guide for Disease Volume X; Epidermolysis Bullosa

Complete Medical Guide for Disease Volume X; 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.
The condition was brought to public attention in the UK through the Channel 4 documentary The Boy Whose Skin Fell Off, chronicling the life and death of Jonny Kennedy, an English man with EB. In the United States, the same could be said of the HBO documentary My Flesh and Blood from 2003.

Complete Medical Guide for Disease Volume X; Epidermolysis Bullosa



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