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[局限性硬皮病(硬斑病)]

[Localized scleroderma (morphea)].

作者信息

Bono Waafa, Dupin Nicolas

机构信息

Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, AP-HP et Université Paris-Descartes, Faculté de Médecine Paris-Descartes, Paris.

出版信息

Presse Med. 2006 Dec;35(12 Pt 2):1923-8. doi: 10.1016/s0755-4982(06)74926-0.

Abstract

UNLABELLED

DEFINITION AND FREQUENCY: Localized scleroderma, also known as morphea, is a sclerotic condition limited to the skin. The specific clinical entity depends on the extent, linear disposition and depth of the lesions. Morphea is ten times more prevalent than systemic sclerosis, and its prognosis is generally good: superficial forms resolve within 3 years.

NO SYSTEMIC INVOLVEMENT

In the absence of symptoms, examinations to detect systemic involvement are purposeless. Plaque morphea is the most frequent clinical presentation. Serious manifestations include extensive morphea that may involve the entire skin or linear forms, especially in children, where they may be severe, especially on the face. There are no immunological markers clearly associated with morphea and no causative agents have been implicated in its pathogenesis, although sclerodermiform dermatitis is reported to be associated with some drugs and toxic agents.

TREATMENT

There is no consensual treatment for morphea. Treatment should be decided according to severity and extent of lesions. Limited lesions may be treated with local steroids such as class IV corticosteroids. Systemic treatment (methotrexate) should be discussed in extensive and linear forms when there is a risk of functional or esthetic complications.

摘要

未标注

定义与发病率:局限性硬皮病,又称硬斑病,是一种局限于皮肤的硬化性疾病。具体的临床类型取决于病变的范围、线状分布及深度。硬斑病的发病率是系统性硬化症的十倍,其预后通常良好:浅表型在3年内可消退。

无全身受累

在没有症状的情况下,检测全身受累情况的检查毫无意义。斑块状硬斑病是最常见的临床表现。严重表现包括可能累及全身皮肤的泛发性硬斑病或线状硬斑病,尤其是在儿童中,这些类型可能较为严重,特别是累及面部时。虽然据报道硬皮病样皮炎与某些药物和有毒物质有关,但目前尚无明确与硬斑病相关的免疫标志物,其发病机制也未明确涉及致病因素。

治疗

对于硬斑病尚无共识性的治疗方法。治疗应根据病变的严重程度和范围来决定。局限性病变可用局部类固醇如IV级皮质类固醇进行治疗。当存在功能或美观并发症风险时,对于泛发性和线状硬斑病应讨论采用全身治疗(甲氨蝶呤)。

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