Paraneoplastic autoimmune multiorgan syndrome (PAMS)

Dario Didona, Giovanni Di Zenzo, Pascal Joly

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
JournalG Ital Dermatol Venereol
DOIs
Publication statusE-pub ahead of print - Oct 16 2020

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