Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure.

Abstract:

:A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. Early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.

journal_name

Oral Oncol

journal_title

Oral oncology

authors

van der Waal RI,Pas HH,Nousari HC,Schulten EA,Jonkman MF,Nieboer C,Stoof TJ,Starink TM,Anhalt GJ

doi

10.1016/s1368-8375(00)00022-1

subject

Has Abstract

pub_date

2000-07-01 00:00:00

pages

390-3

issue

4

eissn

1368-8375

issn

1879-0593

pii

S1368-8375(00)00022-1

journal_volume

36

pub_type

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