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关于脂性渐进性坏死的最新进展:病因、诊断和治疗选择的综述。

Update on necrobiosis lipoidica: a review of etiology, diagnosis, and treatment options.

机构信息

Department of Dermatology, Brown University, Providence, Rhode Island.

Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland.

出版信息

J Am Acad Dermatol. 2013 Nov;69(5):783-791. doi: 10.1016/j.jaad.2013.05.034. Epub 2013 Aug 19.

Abstract

Necrobiosis lipoidica (NL) is a rare chronic granulomatous disease that has historically been associated with diabetes mellitus. Debate exists regarding the etiology and pathogenesis of NL with a widely accepted theory that microangiopathy plays a significant role. NL typically presents clinically as erythematous papules on the front of the lower extremities that can coalesce to form atrophic telangiectatic plaques. NL is usually a clinical diagnosis, but if the clinical suspicion is uncertain, skin biopsy specimen can help differentiate it from sarcoidosis, necrobiotic xanthogranuloma, and granuloma annulare. NL is a difficult disease to manage despite a large armamentarium of treatment options that include topical and intralesional corticosteroids, immunomodulators, biologics, platelet inhibitors, phototherapy, and surgery. Randomized control trials are lacking to evaluate the many treatment methods and establish a standard regimen of care. Disease complications such as ulceration are common, and lesions should also be monitored for transition to squamous cell carcinoma, a less common sequelae.

摘要

脂性坏死性肉芽肿(NL)是一种罕见的慢性肉芽肿性疾病,历史上与糖尿病有关。NL 的病因和发病机制存在争议,目前广泛接受的理论认为微血管病变起着重要作用。NL 临床上通常表现为下肢前部的红色丘疹,可融合形成萎缩性毛细血管扩张性斑块。NL 通常是临床诊断,如果临床怀疑不明确,皮肤活检有助于将其与结节病、坏死性黄色肉芽肿和环状肉芽肿区分开来。尽管有多种治疗方法,包括局部和皮损内皮质类固醇、免疫调节剂、生物制剂、血小板抑制剂、光疗和手术,但 NL 是一种难以治疗的疾病。缺乏随机对照试验来评估许多治疗方法并建立标准的治疗方案。溃疡等疾病并发症很常见,还应监测病变是否转化为鳞状细胞癌,这是一种不太常见的后遗症。

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