lgA pemphigus, intraepidermal neutrophillic type in an 8-year old Filipino female: A case report

ABSTRACT

INTRODUCTION@#lgA pemphigus is a rare, chronic, relapsing, benign group of autoimmune intraepidermal blistering dermatosis with an unknown etiology. It is characterized by significantly pruritic, vesiculopustular lesions that occur mainly on the trunk and proximal extremities. Histopathologic and immunofluorescence studies show intraepidermal blisters and deposition of immunoglobulin A in the intercellular spaces of the epidermis, respectively.@*CASE REPORT@#To our knowledge, we present the first reported pediatric case of lgA pemphigus, intraepidermal neutrophilic type, in an 8-year old Filipino female with a 2-year history of generalized papules and flaccid pustules, some forming an annular pattern. Diagnosis was confirmed by histopathology and direct immunofluorescence. Enzyme-linked immunosorbent assay for Desmoglein 1 was negative. Complete clearance of lesions was achieved with dapsone, colchicine and prednisone.