以特殊皮损为首发临床表现的抗磷脂抗体综合征2例

Two Cases of Antiphospholipid Syndrome( APS) Presenting with Special Skin Manifestation

报告2例以不规则多发性皮肤瘀斑和坏死为首发表现的抗磷脂抗体综合征。例1女,42岁。臀部及下肢大片瘀斑、坏死,血小板减少,抗核抗体、抗心磷脂抗体异常升高,补体降低,皮损组织病理示真皮血管有血栓存在。给予抗凝、血管扩张剂、糖皮质激素、免疫抑制剂和静脉注射用人免疫球蛋白等控制病情的同时,外科手术修复大片坏死皮肤。例2女,47岁。腹部、四肢大片瘀斑、水疱,抗心磷脂抗体异常升高,皮损组织病理示真皮浅层小血管血栓形成。给予糖皮质激素治疗及红光照射,皮损恢复较好。

We reported two cases of antiphospholipid syndrome （APS） presenting with special skin manifestation. Case one：A 42-year-old female suffered from skin ecchymosis and necrosis on buttocks and legs with thrombocyto- penia,hypocomplementemia, and abnormal elevation of ANA and antiphospholipid antibodies （APL）. Histo- pathology revealed thrombosis in dermis and subcutis. With the treatment of anticoagulation,vasodilation,im- munosuppressant, glucocorticoids intravenous immunoglobulin, the diease is in remission. And then the surgi- cal treatment to restored the large area of the necrotic skin was done. Case two ： A 47-year-old female suffered from skin ecchymosis and blister on limbs and abdomen, with abnormal elevation of antiphospholipid antibod- ies. Histopathology revealed thrombosis in superficial dermis. After treatment with glucocorticoids and red light irradiation, the lesion rehabilitation well.