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斯内登综合征及皮肤活检的诊断价值——三名患有脑内病变和网状青斑的年轻患者

Sneddon syndrome and the diagnostic value of skin biopsies - three young patients with intracerebral lesions and livedo racemosa.

作者信息

Legierse Catharina M, Canninga-Van Dijk Marijke R, Bruijnzeel-Koomen Carla A F M, Kuck-Koot Veronica C M

机构信息

Department of Dermatology, Academic Medical Center, Department of Dermatology P.O. Box 22700, 1100 DE Amsterdam, The Netherlands.

出版信息

Eur J Dermatol. 2008 May-Jun;18(3):322-8. doi: 10.1684/ejd.2008.0397. Epub 2008 May 13.

Abstract

Sneddon syndrome is a rare disorder characterised by generalised livedo racemosa of the skin with extracutaneous neurological symptoms like headache, vertigo, transient ischaemic attacks (TIA), stroke, and seizures. Diagnosis of Sneddon syndrome is based on these clinical features and positive findings in skin biopsies, namely the histological proof of occlusion of arterioles by intimal proliferation. We describe three cases of young patients with clinical characteristics of Sneddon syndrome, but in only two cases could this diagnosis be confirmed by skin biopsies. These cases stress the difficulty of diagnosing Sneddon syndrome and show the additive value of skin biopsies in this process.

摘要

斯内登综合征是一种罕见疾病,其特征为皮肤出现全身性网状青斑,并伴有头痛、眩晕、短暂性脑缺血发作(TIA)、中风和癫痫发作等皮肤外神经症状。斯内登综合征的诊断基于这些临床特征以及皮肤活检的阳性结果,即内膜增生导致小动脉闭塞的组织学证据。我们描述了3例具有斯内登综合征临床特征的年轻患者,但只有2例通过皮肤活检确诊。这些病例强调了诊断斯内登综合征的困难,并显示了皮肤活检在此过程中的附加价值。

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