Digital ischemia in Behçet's disease: case-based review

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.