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Differential Diagnosis: Vasculitis, Rheumatoid Arthritis, Behçet’s Disease, and Thromboembolism

  • Benjamin Chaigne
  • Loïc Guillevin
Chapter

Abstract

Although systemic sclerosis is the main systemic cause of digital ulcers, other inflammatory and non-inflammatory conditions are associated with digital ischemia and necrosis. Herein, we review the causes of digital ulcers and distal gangrene, other than systemic sclerosis. Digital ulcers and necrosis arise from three potential mechanisms: vascular inflammation, thrombosis, and vascular spasms. Occurrence and management of digital ulcer in systemic necrotizing vasculitides, rheumatoid arthritis, and Behçet’s disease are specifically addressed. In these three diseases, digital ulcers and distal necrosis are rare and severe manifestations, reflecting vascular injury requiring appropriate management and combining symptomatic and local treatments, immunosuppressants, and biological therapy. Other etiologies and treatments of digital ulcerations, e.g., antiphospholipid syndrome, thromboangiitis obliterans, vascular embolism, or Raynaud’s phenomenon, are also described.

Keywords

Digital ulcer Vasculitis Rheumatoid arthritis Behçet’s disease Thromboembolism Antiphospholipid syndrome Ergotism Raynaud’s syndrome 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Benjamin Chaigne
    • 1
  • Loïc Guillevin
    • 1
  1. 1.National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris DescartesParisFrance

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