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- Title
Epidermolysis Bullosa Simplex Herpetiformis of Dowling-Meara with Mottled Pigmentation: The Relationship Between EBS Herpetiformis and EBS with Mottled Pigmentation.
- Authors
Yong-Kwang Tay; Weston, William L.
- Abstract
A 6-year-old girl with epidermolysis bullosa simplex (EBS) is described. Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, palmoplantar keratoderma, and Improvement with age. An unusual feature was the presence of a striking mottled pigmentation involving the arms, trunk, and legs. Histology, immunofluorescence, and electron microscopy of a fresh lesion showed an intraepidermal split within the basal keratinocytes, focal hyperpigmentation of the basal cells without an inflammatory Infiltrate, and tonofilament clumping. Our patient Illustrates the clinical features of DowlingMeara EBS with the pigmentary changes of EBS with mottled pigmentation. The relationship between EBS of Dowling-Meara and EBS with mottled pigmentation is discussed.
- Subjects
SKIN diseases; PIGMENTATION disorders; BLISTERS; HUMAN skin color; KERATINOCYTES; CELLS; IMMUNOFLUORESCENCE
- Publication
Pediatric Dermatology, 1996, Vol 13, Issue 4, p306
- ISSN
0736-8046
- Publication type
Article
- DOI
10.1111/j.1525-1470.1996.tb01246.x