Category Archives: 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 skin has three layers; the outer layer is called the epidermis the next layer is the dermis, followed by the subcutaneous or hypodermis. In individuals with healthy skin, there are protein anchors, made of collagen, between the layers that prevent them from moving independently from one another (shearing). In people born with EB, the top two skin layers lack the protein anchors that hold them together, and any action that creates friction between them (like rubbing or pressure) will create blisters and painful sores. Sufferers of EB have compared the sores with third-degree burns.
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.
“Butterfly Children” is a term often used to describe younger patients because the skin is said to be as fragile as a butterfly’s wings.
Children with the condition have also been described as “Cotton Wool Babies,” and in South America, “Crystal Skin Children” is the term used.
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