Behçet’s disease is a rare chronic autoimmune disease affecting primarily Middle and East Asian populations between the ages of 20 and 40 years. Behçet’s disease manifests with oral and genital mucocutaneous lesions, ocular disease, venous thrombosis, and central nervous system degradation. Treatment can be challenging and may require immunosuppressive agents and/or topical wound-care. While larger vascular involvement has been reported, digital ischemia due to small-vessel involvement has not been well described in the literature. Based on a systematic literature review, we were only able to find seven published cases of limb ischemia, none of which reported digital involvement. We present a unique case of Behçet’s disease with severe digital ischemia and ulceration caused by small-vessel involvement. The patient was managed successfully with antiplatelet, immunosuppressants, and anti-inflammatories with complete resolution of the ischemic symptoms. By focusing on small-vessel involvement and digital ischemia, we provide insight into clinical presentation and treatment for this very rare vascular manifestation of Behçet’s disease.

中文翻译：

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白塞氏病的数字缺血：基于病例的综述

白塞病是一种罕见的慢性自身免疫性疾病，主要影响20至40岁之间的中亚和东亚人群。白塞氏病表现为口腔和生殖器粘膜皮肤损伤，眼病，静脉血栓形成和中枢神经系统退化。治疗可能具有挑战性，可能需要免疫抑制剂和/或局部伤口护理。尽管已经报道了较大的血管受累，但由于小血管受累引起的数字缺血尚未在文献中得到很好的描述。根据系统的文献综述，我们仅能找到7例公布的肢体缺血病例，但均未报告数字参与。我们提出了由小血管受累引起的严重数字缺血和溃疡性白塞病的一例。使用抗血小板，免疫抑制剂和抗炎药成功治疗了患者，并完全缓解了缺血症状。通过关注小血管受累和数字缺血，我们提供了对这种非常罕见的白塞病血管表现的临床表现和治疗的见解。