A Case of Pemphigus Herpetiformis in a 12-Year-Old Male

Pemphigus herpetiformis (PH) is one of the less common forms of pemphigus. PH in children is unreported. We describe a case of a child who developed PH. Observation. A 12-year-old boy was seen at our department with erosive plaques, vesicles, and crusted cutaneous lesions associated with severe itching persisting for six months. Histologic examination showed an intraepidermal bulla containing rare acantholytic epidermal cells with eosinophilic spongiosis. Direct immunofluorescence demonstrated intercellular Ig G and C3 deposit. The serum titer of antibodies against intercellular epidermal was 1/200？UI/l. Diagnosis of PH was made, and treatment with Dapsone 2？mg/kg per day resulted in total clinical remission. However, two months later, new vesicles reappeared and treatment was begun with prednisone at a dose of 2？mg/kg daily. There was a very good response. Discussion. Childhood pemphigus herpetiformis is a rare disease, often initially misdiagnosed. It must not be forgotten that the disease is a possible cause of erosive mucocutaneous disease in children. 1. Introduction The pemphigus diseases, which include some of the most severe bullous autoimmune skin reactions, are seen predominantly in middle-aged and elderly individuals. Only endemic pemphigus foliaceus in South America most frequently affects juveniles and children. All nonendemic pemphigus diseases, including paraneoplastic pemphigus, have been reported to occur in adolescents and even very rarely in children younger than 10 years. Pemphigus herpetiformis (PH) is considered a variant of pemphigus displaying clinical features similar to dermatitis herpetiformis and a diverse histopathologic pattern with intraepidermal and subcorneal microabscesses, eosinophilic spongiosis, or superficial bullae with usually scant acantholytic cells [1, 2]. The clinical picture is variable, often with coalescent annular or gyrate vesiculopustular lesions. The diagnosis is based on detection of Ig G antikeratinocyte cell surface antibodies, both bound in vivo and in circulation. In the literature, few cases of pemphigus are reported in children. This rarity in childhood may be only apparent as a result of its difficult diagnosis. Pemphigus herpetiformis in a child is unreported. We describe a case that developed PH. 2. Observation An 12-year-old boy was seen at our department in March, 2007, with erosive plaques, vesicles, bulls, and crusted lesions associated with severe itching persisting for six months. The boy’s family history and personal history were unremarkable, and his growth and development had