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Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour
Author(s) -
Hans Wolff,
Christian Kunte,
Gerald Messer,
Klemens Rappersberger,
E Held,
U. Löhrs,
Gerd Plewig,
Michael Meurer
Publication year - 1999
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1999.02669.x
Subject(s) - medicine , pemphigus , paraneoplastic pemphigus , castleman disease , pathology , dermatology , autoantibody , immunology , disease , antibody
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 × 5 × 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.