Back issues No.4 - 1989 / Case Report  

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Epidermolysis Bullosa Atrophicans Generalisata Mitis
Po-Chak Cheng, Boon-Sang Lee, Chwen-Huey Wu, Wen-Lin Lo
Dermatol Sinica 7: 215-226, 1989

A 32-year-old single male has suffered from persistent widespread erythematous papules, vescles and bullae over trunk and extreemities since birth. Sever itching was found before the eruption of lesions. The bullae usually occured with trauma or friction, healed without scarring or milia formation, but resulted in skin atrophy and hypo-pr hyperpigmentation. Besides, nail dystrophy and teeth hypoplasia, urethral stricture were noted. There was no history of consanguinity in family, but one of his younger brother presented with the same clinical manifestation since childhood. Skin biopsy revealed subepidermal cleft. Both direct and indirect immunofluorescence tests showed negative finding. Immunofluorescence staining with anti-type IV collagen antibody showed a linear fluorescent band on the base of the blister. Electron microscopic study proved separation within the lamina lucida, with marked reduce and rudimentary change of hemidesmosome, duplication of basal lamina and vaculoes in basal cells. We believed it to be a typical case of epidermolysis bullosa atrophicans generalisata mitis.

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