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Dystrophic Epidermolysis Bullosa


Group of inherited disorders characterized by mechanically induced blistering occurring below the lamina densa of the basement membrane zone. There is very wide variation in the severity of DEB reflecting the many different mutations affecting the type VII collagen gene. DEB may be inherited as an autosomal dominant or rezessive trait due to a mutation mapped and cloned to chromosome 3 (collagen VII gene). The clinical hallmark of DEB is the tendency for blistered areas to heal with atrophic scars and the development of contractures. In the great majority of cases blister or more often erosion are present very shortly after birth. Digital fusion and « mitten » deformities of hands and feet secondary to repeated episodes of blistering and scarring occur in the severe forms associated with flexural contractures of the knees and wrists. Late complications include the development of epitheliomas, particularly squamous cell carcinomas, in recurrently ulcerated and scarred areas.




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