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Dermatitis Herpetiformis Presenting as Chronic Urticaria
Author(s) -
Powell Gavin R.,
Bruckner Anna L.,
Weston William L.
Publication year - 2004
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.0736-8046.2004.21509.x
Subject(s) - medicine , dermatitis herpetiformis , dermatology , dapsone , enteropathy , asymptomatic , direct fluorescent antibody , skin biopsy , biopsy , pathology , disease , immunology , antibody
Childhood dermatitis herpetiformis (DH) is an immunobullous disease associated with gluten‐sensitive enteropathy. This disease is rare in children and is typically characterized by intensely pruritic vesicles on the extensor surfaces. Definitive diagnosis of DH depends on the direct immunofluorescence finding of granular or fibrillar IgA deposits along the basement membrane zone of biopsied perilesional skin. We report an 11‐year‐old boy with an unusual presentation of DH characterized by a 7‐month history of chronic urticaria‐like skin lesions. He had evanescent, largely asymptomatic, urticarial wheals on his trunk, face, and extremities that were unresponsive to conventional therapy for urticaria. Skin biopsy specimen findings were consistent with DH and direct immunofluorescence of perilesional skin was diagnostic. The patient had no symptoms of gluten‐sensitive enteropathy at the time of diagnosis, and his skin lesions rapidly cleared with dapsone therapy. This patient serves to highlight an unusual presentation of childhood DH and the need to consider this diagnosis when evaluating chronic urticarial lesions in children.