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以泛发性脓疱型银屑病为重点的脓疱型银屑病:分类与诊断标准。一项意大利专家共识。

Pustular psoriasis with a focus on generalized pustular psoriasis: classification and diagnostic criteria. An Italian expert consensus.

作者信息

Costanzo Antonio, Bardazzi Federico, DE Simone Clara, Fabbrocini Gabriella, Foti Caterina, Marzano Angelo V, Naldi Luigi, Parodi Aurora, Prignano Francesca, Girolomoni Giampiero

机构信息

Unit of Dermatology, Department of Biomedical Sciences, Humanitas University, Milan, Italy -

Skin Pathology Laboratory, IRCCS Humanitas Research Hospital, Rozzano, Milan, Italy -

出版信息

Ital J Dermatol Venerol. 2022 Dec;157(6):489-496. doi: 10.23736/S2784-8671.22.07415-1.

Abstract

Generalized pustular psoriasis (GPP) is a severe and life-threatening systemic disease associated with significant morbidity and mortality. Recent progress has been made in understanding the pathogenetic pathways involved in GPP and an intricate interaction between innate and adaptive immune mechanisms has been suggested. Despite formal consensus guidelines on pustular psoriasis currently available in the literature, the definitions and classifications of GPP used across studies were inconsistent. Consequently, there are no unified criteria that can be universally adopted for precise diagnosis, classification and effective treatment of GPP patients with new targeted drugs. The aim of this review was to collect all the main evidence on available diagnostic criteria for GPP and to establish recommendations in order to promote a better stratification and therapeutic management of this severe and heterogeneous disease.

摘要

泛发性脓疱型银屑病(GPP)是一种严重且危及生命的全身性疾病,具有较高的发病率和死亡率。在理解GPP所涉及的发病机制方面已取得了最新进展,并且有人提出先天性和适应性免疫机制之间存在复杂的相互作用。尽管目前文献中有关于脓疱型银屑病的正式共识指南,但各研究中使用的GPP定义和分类并不一致。因此,对于使用新型靶向药物的GPP患者,尚无能够普遍采用的统一标准来进行精确诊断、分类和有效治疗。本综述的目的是收集关于GPP现有诊断标准的所有主要证据,并制定建议,以促进对这种严重且异质性疾病进行更好的分层和治疗管理。

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