Two Cases of Epidermolysis Bullosa Simplex (Dowling-Meara Type).
- Author:
Hyun Joo LEE
1
;
Soon Baek KWON
;
Seok Jong LEE
;
Sang Lip CHUNG
;
Heng Mi KIM
;
Dong Ja KIM
Author Information
1. Department of Dermatology, Kyungpook National University School of Medicine, Taegu, Korea.
- Publication Type:Case Report
- Keywords:
Dowling-Meara type;
Epidermolysis bullosa simplex;
Tonofilament clumping
- MeSH:
Adolescent;
Blister;
Epidermolysis Bullosa Simplex*;
Epidermolysis Bullosa*;
Fingers;
Foot;
Forearm;
Hand;
Humans;
Infant, Newborn;
Intermediate Filaments;
Keratinocytes;
Male;
Toes
- From:Korean Journal of Dermatology
2000;38(7):955-960
- CountryRepublic of Korea
- Language:Korean
-
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.
