Generalized atrophic benign epidermolysis bullosa

Abstract

Eight cases of a new variant of hereditary epidermolysis bullosa (EB), generalized atrophic benign EB, are reported. This is a junctional form of EB that, in contrast to EB letalis of Herlitz, has a good prognosis. It is inherited as an autosomal recessive trait, and the clinical picture is monotonously similar in all patients observed so far, with generalized blister formation, atrophic alopecia, and dystrophic nail changes. Blisters on the skin and mucous membranes heal without scarring or dystrophy but often result in notable atrophy. There is a definite tendency for amelioration of symptoms as the patients age, but therapy has, so far, been ineffective.