IgA pemphigus is a rare neutrophilic acantholytic skin disorder with only approximately 70 cases reported in the indexed literature to date. Here we describe two patients with IgA pemphigus (subcorneal pustular dermatosis type and intraepithelial neutrophilic type) that to our knowledge are the first Scandinavian patients with this disease. Initially, both patients were misdiagnosed as subcorneal pustular dermatosis of Sneddon and Wilkinson and only subsequent careful immunofluorescence studies (in one case with confocal laser scanning microscopy) led to the correct diagnosis. Apart from the expected IgA depositions on epidermal cell surfaces, both patients demonstrated some degree of intercellular IgG-specific immunofluorescence. No circulating IgA autoantibodies were detected. One patient was treated with the standard regime comprising dapsone and prednisolone, whereas in the other case a novel methotrexate and prednisolone combination treatment showed marked clinical efficacy.