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

Rheumatol Int. 2020 Jan;40(1):137-143. doi: 10.1007/s00296-019-04452-z. Epub 2019 Oct 9.

Abstract

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.

Keywords: Behçet’s disease; Digital ischemia; Vasculitis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Antirheumatic Agents / therapeutic use
  • Aspirin / therapeutic use
  • Azathioprine / therapeutic use
  • Behcet Syndrome / drug therapy
  • Behcet Syndrome / pathology
  • Behcet Syndrome / physiopathology*
  • Colchicine / therapeutic use
  • Electromyography
  • Fingers / blood supply
  • Humans
  • Ischemia / drug therapy
  • Ischemia / pathology
  • Ischemia / physiopathology*
  • Male
  • Middle Aged
  • Polyneuropathies / physiopathology
  • Prednisone / therapeutic use
  • Toes / blood supply
  • Tubulin Modulators / therapeutic use
  • Vasculitis / drug therapy
  • Vasculitis / pathology
  • Vasculitis / physiopathology*

Substances

  • Anti-Inflammatory Agents
  • Antirheumatic Agents
  • Tubulin Modulators
  • Azathioprine
  • Aspirin
  • Colchicine
  • Prednisone