Gibson G E, Su W P, Pittelkow M R
Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
J Am Acad Dermatol. 1997 Jun;36(6 Pt 1):970-82. doi: 10.1016/s0190-9622(97)80283-6.
The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for the syndrome are the lupus anticoagulant and anticardiolipin antibodies. Clinical features include recurrent thrombotic events (arterial or venous), repeated fetal loss, and thrombocytopenia. Cutaneous manifestations may occur as the first sign of antiphospholipid syndrome. These include livedo reticularis, necrotizing vasculitis, livedoid vasculitis, thrombophlebitis, cutaneous ulceration and necrosis, erythematous macules, purpura, ecchymoses, painful skin nodules, and subungual splinter hemorrhages. Antiphospholipid syndrome may also be associated rarely with anetoderma, discoid lupus erythematosus, cutaneous T-cell lymphoma, or disorders that closely resemble Sneddon or Degos syndromes. Noninflammatory vascular thrombosis is the most frequent histopathologic feature observed. Prophylaxis and treatment of thrombosis in patients with antiphospholipid syndrome relies principally on anticoagulant and antiplatelet agents.
抗磷脂综合征是一种获得性多系统高凝紊乱疾病，可为原发性或继发于潜在疾病。该综合征的血清学标志物是狼疮抗凝物和抗心磷脂抗体。临床特征包括复发性血栓形成事件（动脉或静脉）、反复流产和血小板减少。皮肤表现可能是抗磷脂综合征的首发症状。这些表现包括网状青斑、坏死性血管炎、类脂质渐进性坏死性血管炎、血栓性静脉炎、皮肤溃疡和坏死、红斑性斑疹、紫癜、瘀斑、疼痛性皮肤结节以及甲下裂片形出血。抗磷脂综合征也可能很少与皮肤松弛症、盘状红斑狼疮、皮肤T细胞淋巴瘤或与斯内登综合征或德戈斯综合征极为相似的疾病相关。非炎性血管血栓形成是最常见的组织病理学特征。抗磷脂综合征患者血栓形成的预防和治疗主要依赖抗凝剂和抗血小板药物。
