Primary antiphospholipid syndrome: two case reports, one with histological examination of skin, peripheral nerve and muscle.

Acta neurologica Pub Date : 1994-06-01
M Macucci, M T Dotti, S Battistini, N De Stefano, V Vecchione, G Orefice, A Malandrini, A Federico
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Abstract

Two cases of primary antiphospholipid antibody syndrome are reported. One patient presented multiple abortions and epilepsy. The second patient was affected by a brain vascular accident, with a residual hemiparesis. Both cases showed livedo reticularis in arms, NMR evidence of diffuse lesions of the white matter, high serum levels of anticardiolipin antibodies and cardiopathy. Lupus anticoagulant was also found in the serum of the first patient, and cortisone and antiaggregants enabled her to reach term in a fifth pregnancy after four miscarriages. In the other case histological examination of specimens of skin, peripheral nerve and skeletal muscle revealed occlusive, non arteriosclerotic vasculopathy and an absence of inflammatory lesions. Histological study has rarely been performed in primary antiphospholipid syndrome but suggests that the mechanism of thrombosis is not vascular; in our subjects it revealed findings similar to those in Sneddon syndrome.

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原发性抗磷脂综合征:2例报告,1例伴有皮肤、周围神经和肌肉的组织学检查。
本文报告2例原发性抗磷脂抗体综合征。1例患者出现多次流产和癫痫。第二例患者受脑血管意外影响,伴有残余性偏瘫。两例均表现为手臂网状增生,核磁共振显示弥漫性白质病变,血清抗心磷脂抗体水平高,并伴有心脏病。在第一位患者的血清中也发现了狼疮抗凝血剂,可的松和抗聚集剂使她在四次流产后第五次怀孕足月。在另一个病例中,皮肤、周围神经和骨骼肌标本的组织学检查显示闭塞性、非动脉硬化性血管病变和无炎性病变。组织学研究很少在原发性抗磷脂综合征中进行,但表明血栓形成的机制不是血管;在我们的研究对象中,它揭示了与斯奈登综合症相似的结果。
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