Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour

A 42-year-old woman presented with oral and labial erosions, conjunctivitis , facial rash and lichenoid erythematous papules on the trunk. Paraneoplast ic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an i ntra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Ca stleman tumour, the skin and mucosal inflammation gradually subsided over t he next 12 months. However due to irreversible pulmonary involvement the pa tient died of intractable respiratory distress 2 years after the onset of t he disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high- dose intravenous immunoglobulins and thalidomide were ineffective. The diag nosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.