Juvenile Bullous Pemphigoid – a Case Report

Bullous pemphigoid is an autoimmune blistering disease that predominantly affects elderly persons and rarely children. We present a 12-year-old girl with sudden appearance of tense blisters on an erythematous base on the trunk, neck, hands and legs with intense pruritus. Standard laboratory test results were within the normal range except for blood eosinophilia of 12% of the total white cell count. Skin biopsy specimens showed evolving subepidermal blisters with perivascular lymphohistiocytic, eosinophil and neutrophil infiltrations in the papillary dermis. Direct immunofluorescence of perilesional skin showed linear, continuous deposits of IgG and C3 along the dermoepidermal junction. Indirect immunofluorescence showed circulating anti-basement membrane zone IgG autoantibodies at a titer of 1:80. We started treatment with systemic corticosteroids, methylprednisolone 0,5 mg/kg per day and 500 mg erythromycin 4 times a day during 10 days. After 3 days 50 mg dapsone (DDS, 4,4-diaminodiphenylsulphone) per day was added. After a few days, there were no new changes on the skin and pruritus disappeared completely.