Letter: Pemphigus herpetiformis of age of onset at 6 years

Pemphigus herpetiformis is a rare entity that combines the clinical features of dermatitis herpetiformis with the immunologic and histological features of pemphigus. It generally affects adults and pediatric cases are uncommon. We report a case of pemphigus herpetiformis in a 6-year-old girl treated successfully with dapsone. A 6-year-old girl presented with a pruritic, erythematous and vesiculous eruption. Histological examination showed acantholysis of the medial and superficial parts of the epidermis. Direct immunofluorescence study revealed cell surface immunoglobulin G and C3 deposits and staining on the dermo-epidermal junction. The child was diagnosed as having pemphigus herpetiformis. Pemphigus herpetiformis produces clinically a pruritic and atypical eruption that resembles dermatitis herpetiformis, with standard histology and immunofluorescence patterns recalling those of pemphigus. Considered to have a good prognosis, it responds well to dapsone and/or to low dose oral corticosteroids. The age range of onset varies from 31 to 81 years according to studies, without a male or female predilection. This is a report of a 6-year-old girl presenting with pemphigus herpetiformis, successfully treated with dapsone for two years with no relapse.