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June 1982

Generalized Atrophic Benign Epidermolysis Bullosa

Author Affiliations

From the Department of Dermatology, University of Innsbruck, Austria. Dr Wolff is now with the University of Vienna.

Arch Dermatol. 1982;118(6):375-384. doi:10.1001/archderm.1982.01650180009008

• 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.

(Arch Dermatol 1982;118:375-384)