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作者报道一例双颊和耳垂部有大片疼痛性皮肤坏死的44岁女性患者,伴齿龈出血,证实体内存在狼疮性抗凝因子。皮损活检,显示真皮血管被含纤维蛋白和血小板组成的血栓阻塞,并且有红细胞外渗,轻度的血管周围浸润,但无血管破坏的证据,其改变与弥漫性血管内凝血或血栓形成性血小板减少性紫癜中所见的相同。前臂正常皮肤直接免疫荧光检查可见基底膜带有IgM沉积,并存在细胞样小体。反复血小板计数轻度降低,高岭土部分凝血激酶时间(PTT)延长,外周血中有纤维蛋白单体,并存在循环IgM复合物,其浓度大于20mg/100ml。异常的高岭土PTT,不能用正常血浆纠正,提示一种抑制因子的存在。检测狼疮性抗凝因子的血小板磷脂中和试验阳性,这种抗凝因子在体外能引起高岭土PTT延长,在体内与血栓形成倾向有关,
The authors report a 44-year-old female with large, painful skin necrosis on the cheek and ear lobes, with bleeding gums, confirming the presence of lupus anticoagulant in the body. Skin lesions biopsy showed clotting of the dermal vessels with fibrin and platelets, extravasation of erythrocytes, mild perivascular infiltration, but no evidence of vascular disruption, which is associated with diffuse intravascular coagulation or thrombosis Thrombocytopenic purpura seen in the same. Direct immunofluorescence examination of normal forearm skin revealed basal lamina with IgM deposition and presence of cell-like bodies. Repeated platelet count was slightly lower, kaolin partial thromboplastin time (PTT) prolongation, fibrin monomer in peripheral blood, and the presence of circulating IgM complex, the concentration greater than 20mg / 100ml. Abnormal kaolin PTT, can not be corrected with normal plasma, suggesting the existence of a suppressor. Detection of lupus anticoagulant factor platelet phospholipid neutralization test positive, this anticoagulant factor in vitro can cause kaolin PTT prolongation in vivo and thrombophilia-related,