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June 1986

The Generalized Atrophic Benign Form of Junctional Epidermolysis Bullosa: Experience With Four Patients in the United States

Author Affiliations

From the Departments of Dermatology, Rush-Presbyterian-St Luke's Medical Center, Chicago (Drs Paller, Kaplan, and Pearson), and University of Alabama at Birmingham School of Medicine, and the Dermatology Section, Medical Service, Birmingham Veterans Administration Medical Center, Birmingham (Dr Fine).

Arch Dermatol. 1986;122(6):704-710. doi:10.1001/archderm.1986.01660180110027

• We encountered four patients in the United States with the generalized atrophic benign form of junctional epidermolysis bullosa (epidermolysis bullosa atrophicans generalisata mitis, nonlethal junctional epidermolysis bullosa). Prior to the performance of definitive diagnostic studies, each patient had been thought for at least a decade to have either a dystrophic or simplex form of epidermolysis bullosa. Each patient had generalized blisters since birth that healed with atrophy and mild scarring but without milia or contractures. Two of the four patients had experienced laryngeal involvement during childhood. In each patient, correct diagnosis was finally established by either electron microscopic examination or immunofluorescence mapping of skin sections from induced blisters.

(Arch Dermatol 1986;122:704-710)