Abstract
A 76-year-old female presented with a 3-year history of erythematous macules and vesicles on her extremities. Since her skin eruptions were not improved by oral administration of prednisolone, she consulted our hospital. She had pigmented macules on her lower extremities, some of which had vesicles around the margins forming a sunflower-like configuration. The mucous membranes were not affected. Histopathological examination of a pustule showed neutrophilic infiltration in the epidermis as well as in the upper dermis. Direct immunofluorescence microscopy of a skin biopsy specimen demonstrated IgA deposition on cell surfaces throughout the epidermis. We diagnosed the patient as having the intraepidermal neutrophilic dermatosis type of IgA pemphigus. A combination of DDS (diaminodiphenylsulfone) 50mg/day and prednisolone 7.5mg/day improved her eruptions. The individual lesions disappeared in a few days leaving hyperpigmentation.