title: [Paraneoplastic pemphigus in a patient with non-Hodgkin lymphoma].
abstract: A 53-year-old man with non-Hodgkin lymphoma developed red, flaky skin, which was initially suggestive of a drug reaction. He also had pneumonia, for which he was admitted for antibiotic treatment. During admission the skin picture changed and blisters and erosions appeared on his body. Skin biopsy and immunological examination led to the diagnosis of paraneoplastic pemphigus (PNP). The patient died five months after the diagnosis of PNP due to PNP pneumonia. PNP is a rare and often aggressive bullous disease with an autoimmune pathogenesis, associated with underlying lymphoproliferative disease. It is characterised by a polymorphous skin rash, painful mucosal erosions, sometimes with respiratory complications due to bronchiolitis obliterans. Diagnosis is based on clinical, histological and immunological findings. The prognosis is unfavourable; death occurs in 90 percent of patients. This case illustrates the importance of histology, immunofluorescence microscopy, and immunoserology in misunderstood skin disorders in patients with lymphoproliferative disease.
authors.affiliation: Martini Ziekenhuis, Afd. interne geneeskunde, Groningen, The Netherlands. ljnwind@hotmail.com
authors.forename: Lynnda J N
authors.initials: LJ
authors.lastname: Wind
authors.forename: Annette W G
authors.initials: AW
authors.lastname: van der Velden
authors.forename: Gilles F H
authors.initials: GF
authors.lastname: Diercks
authors.forename: Hendri H
authors.initials: HH
authors.lastname: Pas
authors.forename: Marcel F
authors.initials: MF
authors.lastname: Jonkman
mesh_terms: D017809:Fatal Outcome; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000150:complications / Q000175:diagnosis*; D010392:Pemphigus / Q000175:diagnosis*; D011014:Pneumonia / Q000175:diagnosis; D011379:Prognosis