Korean J Dermatol.
2000 Jul;38(7):955-960.
Two Cases of Epidermolysis Bullosa Simplex (Dowling-Meara Type)
- Affiliations
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- 1Department of Dermatology, Kyungpook National University School of Medicine, Taegu, Korea.
- 2Departmenet of Pediatrics, Kyungpook National University School of Medicine, Taegu, Korea.
- 3Department of Pathology, Kyungpook National University School of Medicine, Taegu, Korea.
Abstract
- 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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