Atypical neutrophilic dermatosis with subcorneal IgA deposits

A 26-year-old woman had a chronic vesiculopustular and ulcerating skin disease associated with fever and arthritis. Cutaneous biopsy specimens showed an extensive infiltration of the dermis and epidermis by neutrophils. Direct immunofluorescence (IF) revealed linear subcorneal IgA deposits. Indirect IF showed IgA antibodies reactive with the subcorneal zone of normal epidermis. The disease responded to dapsone therapy. The association between neutrophilic dermatoses, including pyoderma gangrenosum, subcorneal pustular dermatosis, and related entities, and IgA involvement, either IgA gammopathies and/or intraepidermal IgA deposits, is emphasized. Intraepidermal IgA deposits are possibly involved in the pathogenesis of our patient's condition and of other cases of unusual neutrophilic dermatoses.