Abstract
IgA pemphigus is an autoimmune intraepidermal vesiculobullous dermatosis that is exceedingly rare in children. We present a case of an 11-year-old boy with a 1-month history of generalized pruritic pustules and painful oral mucosal lesions. Physical examination showed numerous well-demarcated pustules and crusts on the scalp, neck, trunk, lower extremities and genital area. Pustules developed in the center of annular erythematous macules with tiny premature vesicles and pustules at the margin of the annular erythematous macules. Nikolskys sign was negative. The oral cavity showed gingival mucosal erosions without vesicles or pustules. The clinical diagnosis was linear IgA chronic bullous dermatosis of childhood. Differential diagnoses included subcorneal pustular dermatosis and bullous impetigo. Histopathologic examination of the lesion showed supra-basal bulla filled with neutrophils, eosinophils and lymphocytes. The bullous base is lined by a single layer of degenerative basal cells with a few acantholytic cells. Direct immunofluorescence stains of lesional and perilesional skin biopsies both demonstrated deposition of IgA with an intercellular staining pattern homogenous throughout the epidermis and in the hair follicles. There was no deposition of other immunoglobulins examined. The final histopathological diagnosis was IgA pemphigus. The patient was treated with oral steroids along with dapsone and showed excellent recovery. This case highlights clinical and histopathologic features of a rare case of IgA pemphigus in childhood with oral mucosal involvement.
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