title: 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.
authors.affiliation: Department of Dermatology, University Hospital Vrije Universiteit, PO Box 7057, 1007 MB, Amsterdam, The Netherlands. rif.vdwaal@azvu.nl
authors.forename: R I
authors.initials: RI
authors.lastname: van der Waal
authors.forename: H H
authors.initials: HH
authors.lastname: Pas
authors.forename: H C
authors.initials: HC
authors.lastname: Nousari
authors.forename: E A
authors.initials: EA
authors.lastname: Schulten
authors.forename: M F
authors.initials: MF
authors.lastname: Jonkman
authors.forename: C
authors.initials: C
authors.lastname: Nieboer
authors.forename: T J
authors.initials: TJ
authors.lastname: Stoof
authors.forename: T M
authors.initials: TM
authors.lastname: Starink
authors.forename: G J
authors.initials: GJ
authors.lastname: Anhalt
mesh_terms: D000008:Abdominal Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000150:complications* / Q000556:secondary / Q000601:surgery; D008875:Middle Aged; D009059:Mouth Diseases / Q000209:etiology*; D010257:Paraneoplastic Syndromes / Q000209:etiology*; D010392:Pemphigus / Q000209:etiology*; D012131:Respiratory Insufficiency / Q000209:etiology; D017211:Treatment Failure