Pemphigus herpetiformis (PH) is a rare autoimmune blistering disorder that typically presents in adults. However, its occurrence in paediatric patients, especially in very young children, is exceedingly rare. It presents with clinical features resembling dermatitis herpetiformis (DH) and immunologic characteristics similar to pemphigus, belonging to the group of intraepidermal autoimmune bullous diseases. We present the case of a three-year-old female with a history of annular and vesicular lesions on both forearms and legs. A skin biopsy revealed epidermal acanthosis, marked spongiosis, numerous intra-epidermal blisters, and exocytosis of eosinophils and neutrophils. A superficial perivascular lymphocytic infiltrate, accompanied by eosinophils and neutrophils, was also observed in the dermis. The diagnosis was also supported by direct and indirect immunofluorescence. The patient was treated with clobetasol ointment and dapsone, which showed significant improvement in the skin lesions. This case underscores the importance of considering PH in the differential diagnosis of vesicobullous diseases in children and the need for further research to elucidate its pathogenesis and optimal management.
Keywords: an unusual type of pemphigus; herpetiformis; pemphigus; pemphigus herpetiformis; pemphigus in pediatric.
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