REVIEW  PEMPHIGUS Free access

Italian Journal of Dermatology and Venereology 2021 April;156(2):174-83

DOI: 10.23736/S2784-8671.20.06675-4

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

Paraneoplastic autoimmune multiorgan syndrome

Dario DIDONA ¹ ✉, Giovanni DI ZENZO ², Pascal JOLY ³

¹ Department of Dermatology and Allergology, Philipps University, Marburg, Germany; ² Molecular and Cell Biology Laboratory, IDI-IRCCS, Rome, Italy; ³ Department of Dermatology, Rouen University Hospital and INSERM U905, Reference center for autoimmune bullous diseases, Normandie University, Rouen, France

Originally described by Anhalt as paraneoplastic pemphigus in 1990, paraneoplastic autoimmune multiorgan syndrome (PAMS) is a potentially lethal blistering disease, characterized by polymorphous clinical features, including mucocutaneous erosions, blisters, lichenoid papules, and erythemas. Several autoantibodies have been detected in serum of PAMS patients, including antiplakins, anti-alpha-2-macroglobulin like 1, and antidesmogleins autoantibodies. The mortality rate of PAMS is up to 90%. This is due on the one hand to the poor response to treatments and on the other hand to the delay in the diagnosis and to the prognosis of the underlying neoplasia.

KEY WORDS: Autoimmune diseases; Blister; Bronchiolitis obliterans; Desmogleins; Neoplasms; Pemphigus