Epidermolysis Bullosa (EB) is a genetic connective tissue disease causing blisters in the skin and mucous membranes, with an incidence of 1/20,000 live births in the United States. Its severity ranges from mild to lethal.
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 outermost layer is the epidermis, and the middle layer is the dermis. In individuals with healthy skin, there are protein anchors between the layers that prevent them from moving independently from one another (shearing). In people born with EB, these top 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.
“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.
To learn more about the different types and sub-types of Epidermolysis Bullosa please visit Debra.org.
Information provided from wikipedia.org entry.