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[与神经系统疾病相关的皮肤弹性病变和血管异常。附2例报告]

[Cutaneous elastopathy and vascular anomalies associated with neurological disorders. Apropos of 2 cases].

作者信息

Messimy R, Metzger J, Schaison G, Pfister A, Laccourreye H

出版信息

Rev Neurol (Paris). 1975 Jun;131(6):419-31.

PMID:1209057
Abstract

The two cases described exhibited elastopathy associated with arterial stenosis (case 1) or aneurysms (case 2). The first was clearly a case of elastic pseudo-xanthoma with characteristic cutaneous lesions associated with retinal angioid streaks and severe arterial lesions, notably stenosis of the vertebral arteries (especially the left) causing disorders in the mechanism of balance. There were, in addition, mild diabetes, hyperlipidaemia and abnormal cutaneous pigmentation. The second patient, an African, had been hospitalised for an optochiasmatic syndrome which further investigation, notably by arteriography, indicated was caused by compression of the visual pathways due to massive bilateral carotid aneurysms. The patient had molluscoid pseudo-tumours of the axillae and she reported that her father was in the same condition. Although in the first case, cutaneous biopsy enabled diagnosis of systematized elastorrhexis to be confirmed, histological investigation in the second case revealed mainly hyaline sclerosis of the deep dermal layer. The diagnosis of Ehlers-Danlos disease, suggested for this second patient, could not be confirmed. These two cases emphasize the possibility of various neurological disorders occurring in the course of dystrophy of the connectivo elastic tissue.

摘要

所描述的这两个病例表现出与动脉狭窄(病例1)或动脉瘤(病例2)相关的弹性组织病。第一个病例显然是弹性假黄瘤,伴有特征性皮肤病变,与视网膜血管样条纹和严重的动脉病变相关,特别是椎动脉(尤其是左侧)狭窄导致平衡机制紊乱。此外,还有轻度糖尿病、高脂血症和皮肤色素沉着异常。第二个患者是一名非洲人,因视交叉综合征住院,进一步检查,特别是通过动脉造影显示,该综合征是由双侧巨大颈动脉动脉瘤压迫视觉通路所致。该患者腋窝有类软体动物样假肿瘤,她报告说她的父亲也有同样的情况。虽然在第一个病例中,皮肤活检能够确诊系统性弹性组织断裂,但第二个病例的组织学检查主要显示真皮深层透明硬化。为第二个患者提出的埃勒斯-当洛综合征的诊断未能得到证实。这两个病例强调了在结缔组织弹性组织营养不良过程中发生各种神经疾病的可能性。

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