Neumann type pemphigus vegetans with fatal outcome

: We report on a 45-year-old patient, who first developed pemphigus vegetans of the Neumann type 9 years ago and who presented with typical manifestations of the disease 6 years after the first exacerbation. On systemic therapy with corticosteroids progression of the disease ceased. Additional application of azathioprine, cyclophosphamide or gold thiomalate, however, did not make it possible to reduce the high corticosteroid dosage without exacerbation of the disease. The long-term corticosteroid therapy led to sepsis, thrombosis of the leg and pelvic veins and osteoporosis with crush fractures of the vertebrae; corticosteroid therapy was therefore discontinued and replaced by cyclophosphamide bolus therapy associated with gold thiomalate. The subsequent exacerbation of the pemphigus was treated with methotrexate and dapsone. The skin lesions progressed further, however, and the patient died of sepsis 39 weeks after admission to the clinic.