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Cancer Research Frontiers. 2015 Sep; 1(3): 268-279. doi: 10.17980/2015.268

Paraneoplastic autoimmune multi-organ syndrome and oral mucosa involvement: an intriguing disorder

Ioannis Memis1, Dimitrios Andreadis2[*], Ioulianos Apessos3, Eleni Georgakopoulou4, Athanasios Poulopoulos2

1 219-Military Hospital of Didimoteiho, Greece
2 Department of Oral Medicine/Pathology, School of Dentistry, Aristotle University of Thessaloniki, Greece
3 212-Military Hospital of Xanthi, Greece
4 Laboratory of Histology and Embryology National and Kapodistrian University of Athens (NKUA) Greece

 

*Corresponding author: Dr. D. Andreadis DDS, PhD, Assistant Professor. School of Dentistry, Aristotle University of Thessaloniki, Greece, GR-54124. Tel. +302310999538; Email: dandrea@dent.auth.gr

Citation: Memis I, et al. Paraneoplastic autoimmune multi-organ syndrome and oral mucosa involvement: an intriguing disorder. Cancer Research Frontiers. 2015 Sep; 1(3): 268-279. doi: 10.17980/2015.268

Copyright: @ 2015 Ioannis Memis. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Competing Interests: The authors declare that there are no competing interests.

Received August 30, 2015; Revised November 3, 2015; Accepted November 17, 2015.

 

Abstract

Paraneoplastic autoimmune multi-organ syndrome (PAMS) is a life threatening autoimmune disease almost always associated with neoplasia. Patients with this rare disorder present severe blistering and painful erosions of the oral cavity and may exhibit one of a spectrum of at least five clinical variants of the mucocutaneous disease such as pemphigus like, pemphigoid-like, erythema multiforme-like, graft-versus-host disease-like, and lichen planus-like. This syndrome involves multiple organ systems, and its high mortality rate frequently results from constrictive bronchiolitis obliterans. The histologic findings are as diverse as the clinical presentation, often complicating diagnosis, which is typically only confirmed by immuno-dermatologic and serologic laboratory findings. Multiple specific effectors of humoral and cellular autoimmunity mediating epithelial damage have been identified. All the evidence to date on the efficacy of therapeutic modalities has rested on individual case reports, small case series, and expert recommendations. An update of advances in clinical and basic research and management of PAMS is provided in this paper; however, it should be noted that PAMS remains a complex and multidimensional disorder requiring further study to distinguish the precise pathogenic mechanisms and the role of neoplasms in them, the mechanisms behind respiratory complications and the accompanying high rate of mortality, as well as to establish unique diagnostic criteria and more effective treatment procedures.

Key Words: Paraneoplastic Autoimmune Multiorgan Syndrome, paraneoplastic pemphigus

 

 

 

 

 

 

 

 

 

 

 

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