Paraneoplastic pemphigus

G.F.H. Diercks, Hendrikus Pas, Marcel F. Jonkman, Angelique Poot

Research output: Chapter in Book/Report/Conference proceedingChapterAcademic


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.
Original languageEnglish
Title of host publicationAutoimmune bullous diseases
Subtitle of host publicationText and Review
EditorsMarcel Jonkman
PublisherSpringer International Publishing
Number of pages5
ISBN (Electronic)978-3-319-23754-1
ISBN (Print)978-3-319-23753-4
Publication statusPublished - 2016


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

Cite this