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Skin Autoimmunity

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Front. Immunol. | doi: 10.3389/fimmu.2019.01259

Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa

 Jong Hoon Kim1* and Soo-Chan Kim1
  • 1Department of Dermatology, Gangnam Severance Hospital, South Korea

Paraneoplastic pemphigus is a rare but life-threatening mucocutaneous disease mediated by paraneoplastic autoimmunity. Various neoplasms are associated with paraneoplastic pemphigus. 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 paraneoplastic pemphigus. 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 paraneoplastic pemphigus; 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.

Keywords: Paraneoplastic pemphigus, Neoplasms, tolerance, humoral immunity, cell-mediated immunity

Received: 28 Mar 2019; Accepted: 17 May 2019.

Edited by:

Ralf J. Ludwig, Universität zu Lübeck, Germany

Reviewed by:

David A. Fulcher, Australian National University, Australia
Enno Schmidt, Universität zu Lübeck, Germany
Giovanni Di Zenzo, Institute of Dermatology Immaculate (IRCCS), Italy  

Copyright: © 2019 Kim and Kim. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Jong Hoon Kim, Department of Dermatology, Gangnam Severance Hospital, Seoul, South Korea, jhkim074@yuhs.ac