title: Clinical and immunopathological spectrum of paraneoplastic pemphigus.
abstract: BACKGROUND
  Paraneoplastic pemphigus (PNP) is a rare life-threatening autoimmune bullous disorder. The clinical picture is often polymorphous and blisters may be absent. Autoantibodies are directed against several target antigens, including plakins and desmogleins. PNP has a high mortality rate that does not directly depend on the malignancy of the neoplasm. No generally accepted diagnostic criteria for PNP have been defined.
  
  
  METHODS
  On the basis of four selected PNP patients and the relevant literature, the spectrum of clinical and immunopathological findings as well as pathogenesis and treatment options of PNP are presented.
  
  
  RESULTS
  In addition to a neoplasm, severe stomatitis and the presence of autoantibodies against periplakin and envoplakin are characteristic for PNP. Based on the presented data, diagnostic criteria are proposed.
  
  
  CONCLUSIONS
  Knowledge of the polymorphous clinical picture and the complex autoantibody response is essential for an early diagnosis of PNP which has implications for both prognosis and rapid initiation of treatment.
authors.affiliation: Department of Dermatology and Allergology, Bremen Mitte Hospital, Bremen, Germany.
authors.forename: Jutta
authors.initials: J
authors.lastname: Zimmermann
authors.forename: Friedrich
authors.initials: F
authors.lastname: Bahmer
authors.forename: Christian
authors.initials: C
authors.lastname: Rose
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
mesh_terms: D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis* / Q000276:immunology*; D010392:Pemphigus / Q000175:diagnosis* / Q000276:immunology*