title: Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour.
abstract: A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.
authors.affiliation: Dermatologische Klinik und Poliklinik der Ludwig-Maximilians-Universität München, Frauenlobstrasse 9-11, D-80337 Munich, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Wolff
authors.forename: C
authors.initials: C
authors.lastname: Kunte
authors.forename: G
authors.initials: G
authors.lastname: Messer
authors.forename: K
authors.initials: K
authors.lastname: Rappersberger
authors.forename: E
authors.initials: E
authors.lastname: Held
authors.forename: U
authors.initials: U
authors.lastname: Löhrs
authors.forename: G
authors.initials: G
authors.lastname: Plewig
authors.forename: M
authors.initials: M
authors.lastname: Meurer
mesh_terms: D000328:Adult; D005871:Castleman Disease / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008643:Mesentery; D010257:Paraneoplastic Syndromes / Q000209:etiology*; D010392:Pemphigus / Q000209:etiology*; D012131:Respiratory Insufficiency / Q000209:etiology*