title: Paraneoplastic pemphigus in association with hepatocellular carcinoma.
abstract: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous blistering disease associated with neoplasms, most frequently of the lymphoproliferative type. Rare PNP cases related to nonhematological solid tumors have been reported. The patient in this report presented with severe mucocutaneous involvement of PNP associated with hepatocellular carcinoma. Histopathology showed vacuolar interface dermatitis with keratinocyte necrosis and intraepidermal acantholysis. Direct immunofluorescence exhibited deposition of intercellular IgG and complement and granular complement at the dermoepidermal junction. Indirect immunofluorescence testing showed a typical intercellular staining on monkey esophagus and rat bladder epithelium. Immunoprecipitation showed characteristic target antigens of 250, 210, and 190 kDa molecular weights. This patient met all diagnostic criteria for paraneoplastic pemphigus and is, to our knowledge, the first report of a case associated with hepatocellular carcinoma.
authors.affiliation: Department of Dermatology, School of Medicine, University of Vienna, Austria. gabriele.hinterhuber@akh-wien.ac.at
authors.forename: Gabriele
authors.initials: G
authors.lastname: Hinterhuber
authors.forename: Johannes
authors.initials: J
authors.lastname: Drach
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Riedl
authors.forename: Kornelia
authors.initials: K
authors.lastname: Böhler
authors.forename: Peter
authors.initials: P
authors.lastname: Ferenci
authors.forename: Klaus
authors.initials: K
authors.lastname: Wolff
authors.forename: Dagmar
authors.initials: D
authors.lastname: Foedinger
mesh_terms: D000368:Aged; D006528:Carcinoma, Hepatocellular / Q000150:complications* / Q000601:surgery; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000601:surgery; D009367:Neoplasm Staging; D010257:Paraneoplastic Syndromes / Q000175:diagnosis*; D010392:Pemphigus / Q000175:diagnosis* / Q000209:etiology; D035583:Rare Diseases; D018570:Risk Assessment