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Butterfly Skin Disease

Butterfly skin disease is the name given to a group of rare genetic disorders characterized by skin that is as fragile as butterfly wings. The medical name for these inherited disorders is epidermolysis bullosa, or EB. EB is believed to affect 25,000 to 50,000 people in the United States. There are four main types of epidermolysis bullosa: * Dystrophic epidermolysis bullosa * Epidermolysis bullosa simplex * Hemidesmosomal epidermolysis bullosa * Junctional epidermolysis bullosa Another rare type of epidermolysis bullosa, called epidermolysis bullosa acquisita, is an autoimmune disorder. It may be difficult to tell this condition apart from another autoimmune skin disorder called mucous membrane pemphigoid. Depending on the type of epidermolysis bullosa, the condition can vary from minor blistering of the skin to a lethal form involving other organs. The condition generally starts at birth or soon after. Epidermolysis bullosa acquisita, however, usually appears in adults over age 50, although it can occur in children. Also, mild cases of epidermolysis bullosa simplex may remain undiagnosed until adulthood. Identifying the exact type of epidermolysis bullosa is complicated. Even within the main types listed above, there are many subtypes. For example, Weber-Cockayne is the most common form of epidermolysis bullosa simplex. This type involves blistering of the palms and soles and may include excessive sweating. Epidermolysis bullosa is a usually inherited blistering disease of the skin and mouth membranes. There are more than 25 subtypes of the butterfly disease and the more severe the involvement the earlier will blisters occur, usually from mild trauma such as that encountered when a baby crawls or lifts objects or goes through teething. Severe cases may be fatal from fluid loss and infection of the open wounds which form after the blisters break, but most cases are relatively mild. The basic defect is usually that of a poor connection between the skin/membrane and the underlying connective tissues. This problem can also be acquired later in life if antibodies form against those connecting fibers. There is no treatment available beyond avoidance of trauma, replacement of lost body fluids and antibiotic treatment of resulting infections.

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