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| Definition: | A group of inherited disorders in which massive blistering of the skin develops in response to minor trauma.
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| Alternative Names: | Epidermolysis bullosa simplex; Epidermolysis bullosa letalis; Weber-Cockayne syndrome; Dominant and recessive epidermolysis bullosa
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| Causes, incidence, and risk factors: | Epidermolysis bullosa consists of several different conditions, varying in severity from minor blistering to a lethal form in which the constant massive blistering and scarring ultimately lead to death. These conditions are frequently inherited.
The hallmark of these conditions is the formation of large, fluid-filled blisters that develop in response to minor trauma. Some infants may have large blisters at birth. Others start shortly after birth. Chafing (wearing away) of the skin, rubbing, or even increased room temperature may cause blisters to form.
In the severe forms, scarring after blister formation may cause deformities, fusion of the fingers and toes, contracture deformities (as at the fingers, elbows and knees) and mouth and esophagus scarring that leads to feeding and swallowing difficulties. Secondary infection is common.
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Review Date: 12/1/2001
Reviewed By: Michael Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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