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局限性和系统性硬皮病。

Localized and systemic scleroderma.

作者信息

Hawk A, English J C

机构信息

Department of Dermatology, University of Virginia School of Medicine, Charlottesville 22908-0718, USA.

出版信息

Semin Cutan Med Surg. 2001 Mar;20(1):27-37. doi: 10.1053/sder.2001.23093.

Abstract

Scleroderma is a broad term encompassing both localized and systemic sclerosis. Localized scleroderma is a cutaneous limited fibrosis that manifests as plaque morphea, generalized morphea, linear scleroderma, and deep morphea. Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease. Limited systemic sclerosis is typically preceded by Raynaud's phenomenon, involves cutaneous sclerosis distal to the elbows, with gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. Diffuse systemic scleroderma is characterized by simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, and positive serology for antitopoisomerase and anti-RNAP III antibodies. This article discusses the classification, epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of the scleroderma.

摘要

硬皮病是一个广义术语,涵盖局限性和系统性硬化症。局限性硬皮病是一种皮肤局限性纤维化,表现为斑块状硬斑病、泛发性硬斑病、线状硬皮病和深部硬斑病。系统性硬皮病(硬化症)可表现为局限性或弥漫性疾病。局限性系统性硬化症通常先出现雷诺现象,累及肘部远端的皮肤硬化,伴有胃肠道和肺部纤维化,且抗着丝点抗体阳性。弥漫性系统性硬皮病的特征是同时出现雷诺现象,肘部近端皮肤受累,伴有胃肠道、肺部、肾脏和心脏纤维化,抗拓扑异构酶和抗RNA聚合酶III抗体血清学检查呈阳性。本文讨论了硬皮病的分类、流行病学、发病机制、临床表现、治疗及预后。

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