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泛发性脓疱型银屑病(von Zumbusch)。

Generalized pustular psoriasis (von Zumbusch).

机构信息

Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil.

Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil.

出版信息

An Bras Dermatol. 2022 Jan-Feb;97(1):63-74. doi: 10.1016/j.abd.2021.05.011. Epub 2021 Nov 24.

Abstract

Generalized pustular psoriasis (von Zumbusch) is a rare and acute eruption characterized by multiple sterile pustules over an erythematous and edematous background, eventually associated with psoriasis vulgaris. Classically, it manifests as a potentially severe systemic picture and demands prompt diagnosis and intervention. The duration of each flare-up and intervals between the pustular episodes is extremely variable. Recently, genetic abnormalities have been identified mainly in the familial and early variants of this disease. The therapeutic arsenal is limited; however, new drugs being evaluated aim to control both pustular flare-ups and disease recurrences.

摘要

泛发性脓疱型银屑病(von Zumbusch)是一种罕见的急性发疹性疾病,其特征为在红斑和水肿的背景上出现多个无菌脓疱,最终可发展为寻常型银屑病。临床上,该病表现为一种潜在的严重全身疾病,需要及时诊断和干预。每次发作的持续时间和脓疱发作之间的间隔时间差异极大。最近,主要在该疾病的家族性和早期变异型中发现了遗传异常。治疗方法有限;然而,正在评估的新药旨在控制脓疱发作和疾病复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ca34/8799844/38257d366dda/gr1.jpg

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