H. Wolff et al., Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour, BR J DERM, 140(2), 1999, pp. 313-316
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.