Paraneoplastic pemphigus: Paraneoplastic autoimmune disease of the skin and mucosa

Jong Hoon Kim, Soo Chan Kim

Research output: Contribution to journalReview articlepeer-review

48 Citations (Scopus)


Paraneoplastic pemphigus (PNP) is a rare but life-threatening mucocutaneous disease mediated by paraneoplastic autoimmunity. Various neoplasms are associated with PNP. Intractable stomatitis and polymorphous cutaneous eruptions, including blisters and lichenoid dermatitis, are characteristic clinical features caused by humoral and cell-mediated autoimmune reactions. Autoreactive T cells and IgG autoantibodies against heterogeneous antigens, including plakin family proteins and desmosomal cadherins, contribute to the pathogenesis of PNP. Several mechanisms of autoimmunity may be at play in this disease on the type of neoplasm present. Diagnosis can be made based on clinical and histopathological features, the presence of anti-plakin autoantibodies, and underlying neoplasms. Immunosuppressive agents and biologics including rituximab have been used for the treatment of PNP; however, the prognosis is poor due to underlying malignancies, severe infections during immunosuppressive treatment, and bronchiolitis obliterans mediated by autoimmunity. In this review, we overview the characteristics of PNP and focus on the immunopathology and the potential pathomechanisms of this disease.

Original languageEnglish
Article number1259
JournalFrontiers in Immunology
Issue numberJUN
Publication statusPublished - 2019

Bibliographical note

Funding Information:
This work was supported by the National Research Foundation Grants (NRF-2018R1D1A1B07045532).

Publisher Copyright:
© 2019 Kim and Kim.

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology


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