副肿瘤自身免疫多器官综合征(PAMS)

IF 2 Q3 Medicine

Giornale Italiano Di Dermatologia E Venereologia Pub Date : 2020-10-16 DOI:10.23736/S0392-0488.20.06675-4

D. Didona, G. Di Zenzo, P. Joly

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引用次数: 3

摘要

副肿瘤自身免疫性多器官综合征(PAMS)最初由Anhalt在1990年描述为副肿瘤天疱疮，是一种潜在的致死性水疱疾病，以多形态临床特征为特征，包括皮肤粘膜糜烂、水疱、地衣样丘疹和红斑。PAMS患者血清中检测到多种自身抗体，包括抗血小板抗体、抗α -2-巨球蛋白样1抗体和抗粘粒蛋白自身抗体。PAMS的死亡率高达50%。这一方面是由于对治疗的反应较差，另一方面是由于诊断和潜在肿瘤的预后延迟。

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Paraneoplastic autoimmune multiorgan syndrome (PAMS).

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.

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来源期刊

Giornale Italiano Di Dermatologia E Venereologia DERMATOLOGY-

CiteScore

1.90

自引率

0.00%

发文量

审稿时长

6-12 weeks

期刊介绍： The journal Giornale Italiano di Dermatologia e Venereologia publishes scientific papers on dermatology and sexually transmitted diseases. Manuscripts may be submitted in the form of editorials, original articles, review articles, case reports, therapeutical notes, special articles and letters to the Editor. Manuscripts are expected to comply with the instructions to authors which conform to the Uniform Requirements for Manuscripts Submitted to Biomedical Editors by the International Committee of Medical Journal Editors (www.icmje.org). Articles not conforming to international standards will not be considered for acceptance.