斯内登综合征：17例患者经皮肤活检和磁共振成像诊断

Sneddon's syndrome: diagnosis by skin biopsy and MRI in 17 patients.

作者信息

Stockhammer G, Felber S R, Zelger B, Sepp N, Birbamer G G, Fritsch P O, Aichner F T

机构信息

Department of Neurology, University of Innsbruck, Austria.

出版信息

Stroke. 1993 May;24(5):685-90. doi: 10.1161/01.str.24.5.685.

DOI:10.1161/01.str.24.5.685

PMID:8488523

Abstract

BACKGROUND AND PURPOSE

Sneddon's syndrome, characterized by generalized livedo racemosa and cerebrovascular lesions, is an underdiagnosed disease. We evaluated clinical, laboratory, histological, and neuroradiological findings in a series of 17 patients to improve diagnostic criteria for Sneddon's syndrome.

METHODS

Patients with generalized livedo racemosa and cerebrovascular events were included in the study. All underwent neurological and dermatological examination, skin biopsy, computed tomographic scan, magnetic resonance imaging as well as magnetic resonance angiography, sonography of the extracranial arteries, and a comprehensive laboratory protocol.

RESULTS

Completed stroke was present in eight patients, and 15 reported transient neurological deficits. Magnetic resonance imaging yielded cerebral abnormalities in 16 of 17, whereas computed tomographic scans were abnormal in only 12 of 16 patients. Magnetic resonance imaging revealed more lesions in individual patients than did computed tomography. Magnetic resonance angiography demonstrated patent intracranial vessels in 16 of 17 patients. Skin biopsy showed distinct histopathological findings in all patients. The involved vessels were small to medium-sized arteries at the border between dermis and subcutis. Early inflammatory reactions were followed by subendothelial proliferation and a late fibrotic stage. Laboratory examinations showed impaired creatinine clearance in eight patients, whereas all other laboratory tests, including antiphospholipid antibodies, were normal.

CONCLUSIONS

In this series, magnetic resonance imaging and skin biopsy were useful for confirmation of the diagnosis of Sneddon's syndrome. Magnetic resonance findings were not specific, but the high sensitivity for detection of asymptomatic brain lesions helped to confirm the diagnosis in patients with transient symptoms. Histological features of skin biopsies were characteristic if appropriate techniques were employed.

摘要

背景与目的

斯内登综合征以全身性网状青斑和脑血管病变为特征，是一种诊断不足的疾病。我们评估了17例患者的临床、实验室、组织学和神经放射学检查结果，以完善斯内登综合征的诊断标准。

方法

全身性网状青斑和脑血管事件患者纳入本研究。所有患者均接受了神经和皮肤科检查、皮肤活检、计算机断层扫描、磁共振成像以及磁共振血管造影、颅外动脉超声检查和全面的实验室检查方案。

结果

8例患者出现完全性卒中，15例报告有短暂性神经功能缺损。17例中有16例磁共振成像显示脑部异常，而16例患者中只有12例计算机断层扫描异常。磁共振成像显示的个体患者病变比计算机断层扫描更多。17例患者中有16例磁共振血管造影显示颅内血管通畅。所有患者皮肤活检均显示出明显的组织病理学表现。受累血管为真皮与皮下组织交界处的中小动脉。早期炎症反应后出现内皮下增生和晚期纤维化阶段。实验室检查显示8例患者肌酐清除率受损，而所有其他实验室检查，包括抗磷脂抗体，均正常。