Pemphigus is an uncommon mucocutaneous disease caused by autoantibodies against desmosomal antigens. Juvenile cases are rare. The diagnosis is often delayed due to confusion with other entities.

The biopsy performed for our patient showed suprabasal acantholysis. Direct immunofluorescence (DIF) studies of frozen skin tissue showed positive intercellular staining for IgG within the epidermis. Blood samples for indirect immunofluorescence (IIF) on rabbit’s esophagus demonstrated circulating IgG autoantibodies at a titer of 1:100.

A diagnosis of juvenile pemphigus vulgaris (PV) was made. Treatment was started with 1 mg/kg/d (50 mg/d) methylprednisolone. A gradual remission was observed. Complete regression of the lesion was obtained after 30 days, and the dose of methylprednisolone was gradually reduced after 45 days without any recurrence. No side effects were observed.

After 3 years the maintenance 5 mg/d methylprednisolone treatment was discontinued, and the girl has had no relapses over the last 7 years.

Pemphigus is a group of autoimmune blistering skin disease characterized by blister formation. Blisters are due to the loss of keratinocyte cell-cell adhesion in the superficial and deep epidermis [1].