Reagin Heather, Marks Etan, Weis Stephen, Susa Joseph
University of North Texas Health Science Center, Fort Worth, TX.
Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY.
Am J Dermatopathol. 2018 Sep;40(9):682-685. doi: 10.1097/DAD.0000000000001133.
We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Livedoid vasculopathy most commonly affects the distal lower extremities and is characterized by irregular, punched-out, painful ulcers that heal with stellate white scars of atrophie blanche. Histologically, it reveals segmental hyalinizing vessels, focal thrombosis, and endothelial proliferation. The etiology is currently unclear, but it has been shown to be related to procoagulant states and a diagnosis of livedoid vasculopathy should prompt a thorough hypercoagulable workup, including testing for SCD in high-risk patients.
我们报告了一例病例，一名43岁有镰状细胞病（SCD）病史的非裔美国男性出现了一个不愈合的溃疡。活检显示为类脂质渐进性坏死性血管病的特征。此前，类脂质渐进性坏死性血管病仅在一名有镰状细胞性状的患者中被描述过，但从未在SCD患者中出现过。类脂质渐进性坏死性血管病最常累及下肢远端，其特征为不规则、凹陷、疼痛性溃疡，愈合后留下白色萎缩性星状瘢痕。组织学上，它表现为节段性透明变性血管、局灶性血栓形成和内皮细胞增生。其病因目前尚不清楚，但已证明与促凝状态有关，类脂质渐进性坏死性血管病的诊断应促使进行全面的高凝检查，包括对高危患者进行SCD检测。
