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Candidate SCOPUS
Dowling-Meara형 단순성 수포성 표피박리증 2 예
Two Cases of Epidermolysis Bullosa Simplex ( Dowling-Meara Type )
이현주(Hyun Joo Lee),권순백(Soon Baek Gwon),이석종(Suk Jong Lee),정상립(Sang Rib Jung),김행미(Haeng Mi Kim),김동자(Dong Ja Kim)
UCI I410-ECN-0102-2009-510-004771732

The Dowling-Meara type of epidermolysis bullosa simplex is a rare genodermatosis transmitted by autosomal dominant pattern. It starts during the first 3 months of life and is characterized by generalized bullae in a herpetiform pattern. We report two cases of Dowling-Meara type of epidermolysis bullosa simplex. The first case is an 18-year-old male patient who showed bullae in a herpetiform arrangement on the dorsa of both hands, forearms, and trunk, hyperkeratosis on the palms and soles, postinflammatory hypo- and hyperpigmentations, and dystrophy of finger and toe nails. The second case is a 1-month-old boy who showed multiple bullae in a herpetiform and confluent arrangement scattered on the whole body, particularly on the dorsa of both feet and hands. Microscopically and electro-microscopically, two cases all showed intraepidermal blisters formed within the basal keratinocyte in association with clumping of the tonofilaments and cytolysis of the basal cells.

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