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一例抗核抗体和抗心磷脂抗体阳性的斯内登综合征:原发性抗磷脂综合征?

A case of Sneddon's syndrome with positive ANA and anti-cardiolipin antibodies: primary anti-phospholipid syndrome?

作者信息

Otoyama K, Katayama I, Suzuki Y, Tone T, Nishioka K, Nishiyama S

机构信息

Department of Dermatology, Kitasato University School of Medicine, Sagamihara, Japan.

出版信息

J Dermatol. 1990 Aug;17(8):489-92. doi: 10.1111/j.1346-8138.1990.tb01681.x.

Abstract

A 22-year-old woman developed ulcerative lesions on the lower extremities which usually exacerbated during the summer. Histological analysis revealed a micro-thrombotic lesion in the deep dermis without inflammatory cell infiltration or fibrinoid degeneration of blood vessels. Magnetic resonance imaging revealed multiple cerebral infarctions. Abnormal laboratory findings included an elevated anti-cardiolipin antibody titer and positive speckled pattern ANA (x80), but without other manifestations or signs of SLE. FACS analysis revealed that the patient's serum reacted with ethanol fixed endothelial cells in addition to keratinocytes and peripheral blood neutrophils. This case was thought to be livedo reticularis and cerebral thrombotic lesions (Sneddon's syndrome) associated with atrophie blanche or livedo(id) vasculitis and may be one clinical subset of primary anti-phospholipid syndrome.

摘要

一名22岁女性下肢出现溃疡性病变,通常在夏季加重。组织学分析显示真皮深层有微血栓形成病变,无炎症细胞浸润或血管纤维蛋白样变性。磁共振成像显示多发性脑梗死。实验室检查异常包括抗心磷脂抗体滴度升高和斑点型抗核抗体阳性(x80),但无系统性红斑狼疮的其他表现或体征。流式细胞术分析显示,患者血清除了与角质形成细胞和外周血中性粒细胞反应外,还与乙醇固定的内皮细胞反应。该病例被认为是与白色萎缩或青斑样(特发性)血管炎相关的网状青斑和脑血栓形成病变(斯内登综合征),可能是原发性抗磷脂综合征的一种临床亚型。

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