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.
An 18-year-old man presented with erythematous lesions over the nose of 1 year's duration without sensitivity to sunlight. He was otherwise well and there was no relevant family history. Physical examination showed excessive sweating of the nose, cheeks, and upper lip, and erythema of the nose covered by beads of sweat and vesicles (Fig. 1). The rest of the physical examination was normal. The skin biopsy showed a discrete mononuclear cell perivascular infiltrate.
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