Paraneoplastic Pemphigus

  • Angelique M. PootEmail author
  • Gilles F. H. Diercks
  • Hendri H. Pas
  • Marcel F. Jonkman


Paraneoplastic pemphigus is a rare but severe autoimmune disease characterized by severe stomatitis and a variety of cutaneous manifestations in association with an underlying neoplasia. Pulmonary involvement may also occur. The pathogenesis involves the production of autoantibodies against desmogleins, plakins, and the protease inhibitor alpha-2-macroglobulin-like 1, but T-cell-mediated autoimmunity is also thought to play a role. Diagnosis usually relies on the demonstration of a specific subset of circulating autoantibodies in patient serum, although in a small subset of patients, these autoantibodies might be absent. Due to its rarity, there are no set of guidelines for treatment. The general approach includes a variety of immunosuppressive agents and treatment of the underlying neoplasia. Despite treatment, paraneoplastic pemphigus has high mortality rates, often due to sepsis, respiratory failure, or progression of the underlying malignancy.


Autoimmune disease Pemphigus Paraneoplastic Plakins Alpha-2-macroglobulin-like 1 Stomatitis Neoplasia Paraneoplastic autoimmune multiorgan syndrome 


  1. 1.
    Anhalt GJ. Paraneoplastic pemphigus. J Investig Dermatol Symp Proc. 2004;9:29–33.CrossRefPubMedGoogle Scholar
  2. 2.
    Czernik A, Camilleri M, Pittelkow MR, Grando SA. Paraneoplastic autoimmune multiorgan syndrome: 20 years after. Int J Dermatol. 2011;50:905–14.CrossRefPubMedGoogle Scholar
  3. 3.
    Leger S, Picard D, Ingen-Housz-Oro S, Arnault JP, Aubin F, Carsuzaa F, Chaumentin G, Chevrant-Breton J, Chosidow O, Crickx B, D’incan M, Dandurand M, Debarbieux S, Delaporte E, Dereure O, Doutre MS, Guillet G, Jullien D, Kupfer I, Lacour JP, Leonard F, Lok C, Machet L, Martin L, Paul C, Pignon JM, Robert C, Thomas L, Weiller PJ, Ferranti V, Gilbert D, Courville P, Houivet E, Benichou J, Joly P. Prognostic factors of paraneoplastic pemphigus. Arch Dermatol. 2012;148:1165–72.CrossRefPubMedGoogle Scholar
  4. 4.
    Zimmermann J, Bahmer F, Rose C, Zillikens D, Schmidt E. Clinical and immunopathological spectrum of paraneoplastic pemphigus. J Dtsch Dermatol Ges. 2010;8:598–606.PubMedGoogle Scholar
  5. 5.
    Poot AM, Diercks GF, Kramer D, Schepens I, Klunder G, Hashimoto T, Borradori L, Jonkman MF, Pas HH. Laboratory diagnosis of paraneoplastic pemphigus. Br J Dermatol. 2013;169:1016–24.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Angelique M. Poot
    • 1
    Email author
  • Gilles F. H. Diercks
    • 2
  • Hendri H. Pas
    • 1
  • Marcel F. Jonkman
    • 1
  1. 1.Department of DermatologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands
  2. 2.Departments of Pathology and Medical BiologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands

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