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Epidermolysis Bullosa Simplex Herpetiformis of Dowling‐Meara with Mottled Pigmentation: The Relationship Between EBS Herpetiformis and EBS with Mottled Pigmentation
Author(s) -
Tay YongKwang,
Weston William L.
Publication year - 1996
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1996.tb01246.x
Subject(s) - dermatitis herpetiformis , medicine , dermatology , epidermolysis bullosa simplex , epidermolysis bullosa , pathology , disease
A 6–year‐oid girl with epidermolysis bullosa simplex (EBS) is described. Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, palmo‐plantar 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 kerati‐nocytes, focal hyperpigmentation of the basal cells without an inflammatory infiltrate, and tonofilament clumping. Our patient illustrates the clinical features of Dowling‐Meara EBS with the pigmentary changes of EBS with mottled pigmentation. The relationship between EBS of Dowling‐Meara and EBS with mottled pigmentation is discussed.