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Giornale Italiano di Dermatologia e Venereologia 2020 Oct 16

DOI: 10.23736/S0392-0488.20.06675-4

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

Paraneoplastic autoimmune multiorgan syndrome (PAMS)

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, Centre de Référence des Maladies Bulleuses Autoimmunes, Normandie University, Rouen, France


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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 erythema. Several autoantibodies have been detected in serum of PAMS patients, including anti-plakins, anti-alpha-2-macroglobulin like 1, and anti-desmogleins autoantibodies. The mortality rate of PAMS is up to 50%. 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 blistering disease; Bronchiolitis obliterans; Desmoglein; Malignancy; Pemphigus

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