Introduction: Intraepidermal IgA pustulosis is a vesiculopustular dermatosis defined by pemphigus type intercellular deposit exclusively of IgA. It is a member of the pemphigus group and may be related to neurtrophilic dermatoses.
Case report: A child had vesiculopustular lesions of the limbs since the age of 11 years. Biopsy showed the subcorneal intraepidermal nature of the pustules and exclusive IgA deposit throughout the epiderma. Indirect immunofluorescence and protein immunoelectrophoreses were negative. Immunotransfer to beef tongue extract evidenced a 150-160 kDa band corresponding to IgA and IgG desmoglein. Treatment with general corticosteroids followed by pristinamycin was successful.
Discussion: This is the first case report showing evidence of antiepidermal antibodies directed against the superficial pemphigus antigen.