A childhood case with pemphigus herpetiformis

Pemphigus herpetiformis is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Here, we described a 13-year-old-boy who presented with mild pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk and extremities. Mucous membranes were intact. Tzanck smear showed acantholytic cells. Histopathological examination of lesions revealed subcorneal acantholysis and neutrophilic infiltration. Direct immunofluorescence examination demonstrated IgG and C3 deposition on keratinocyte cell surfaces, and indirect immunofluorescence examination showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1: 20. Based on clinical and laboratory findings, pemphigus herpetiformis was diagnosed. This case is found worth to be presented for both displaying a rare form of pemphigus and for the relatively low incidence of the disease in childhood.

Pemfigus herpetiformisli bir çocuk olgu

Pemfigus herpetiformis klinik olarak herpetiform daðýlým gösteren eritemli veya ürtikeryal plaklar ve veziküller ile karakterize nadir görülen bir pemfigus varyantýdýr. Burada, gövde ve ekstremitelerinde hafif kaþýntýlý, annüler eritem ve herpetiform dizilim gösteren vezikülleri olan, 13 yaþýnda bir erkek çocuk sunulmuþtur. Mukozalarý tutmayan ve Tzanck yaymada akantolitik hücreler gösteren lezyonlarýn histopatolojik incelemesinde subkorneal akantoliz ve nötrofil infiltrasyonu gözlendi. Direkt immünofloresan incelemede intersellüler aralýkta IgG ve C3 depolanmasý, indirekt immünofloresan incelemede ise 1/20 dilüsyonda keratinosit hücre yüzeyine karþý dolanan IgG antikorlarý saptandý. Bu bulgularla pemfigus herpetiformis tanýsý alan olgu, hem pemfigusun nadir bir formuna sahip olmasý hem de bu hastalýðýn görülme sýklýðý açýsýndan nadir bir yaþ grubuna dahil olmasý nedeniyle sunulmaya deðer görülmüþtür.