Internal Medicine Department, Thrombosis and Vasculitis Unit, Complexo Hospitalario Universitario de Vigo, Spain, Faculty of Medicine, University of Santiago de Compostela, Spain.
Lupus. 2010 Oct;19(11):1340-3. doi: 10.1177/0961203310373783. Epub 2010 Jul 21.
Livedoid vasculopathy is a rare condition which predominantly affects young women. It is characterized by intense painful purpuric maculae in the legs, ankles and feet, due to thrombosis of the small and medium-sized dermal vessels, in the absence of vasculitis. Livedoid vasculopathy has been frequently associated with hypercoagulable states and antiphospholipid syndrome. We describe a 34-year-old White woman suffering from systemic lupus erythematosus, livedo reticularis, haemolytic anaemia, severe thrombocytopenia and recurrent venous thrombosis who was admitted to the hospital for extremely painful purpuric lesions in her lower limbs. The clinical and histological findings were diagnostic of livedoid vasculopathy. Once the initial sub-therapeutic international normalized ratio levels were corrected, livedoid vasculopathy did not recur. Tests for antiphospholipid antibodies were repeatedly negative. This case, the first reported of livedoid vasculopathy in a patient with seronegative antiphospholipid syndrome and systemic lupus erythematosus, draws attention to livedoid vasculopathy, a thrombotic dermopathy that may be under-diagnosed in patients with antiphospholipid syndrome.
皮肤白细胞破碎性血管炎是一种罕见的疾病，主要影响年轻女性。其特征为腿部、脚踝和足部出现剧烈疼痛性瘀斑，这是由于小及中等大小的真皮血管血栓形成，无血管炎。皮肤白细胞破碎性血管炎常与高凝状态和抗磷脂综合征相关。我们描述了一位 34 岁白人女性，患有系统性红斑狼疮、网状青斑、溶血性贫血、严重血小板减少和复发性静脉血栓形成，因下肢极度疼痛性瘀斑入院。临床和组织学检查结果诊断为皮肤白细胞破碎性血管炎。一旦初始国际标准化比值低于治疗范围的水平得到纠正，皮肤白细胞破碎性血管炎就不再复发。抗磷脂抗体检测反复呈阴性。这是首例报道的抗磷脂抗体阴性的系统性红斑狼疮伴皮肤白细胞破碎性血管炎患者，该病例提醒人们注意皮肤白细胞破碎性血管炎，这种血栓性皮肤病在抗磷脂综合征患者中可能诊断不足。
