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

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Atlas of Ulcers in Systemic Sclerosis

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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.

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Authors and Affiliations

  1. National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Descartes, Paris, France

    Benjamin Chaigne & Loïc Guillevin

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  1. Benjamin Chaigne
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  2. Loïc Guillevin
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  1. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

    Marco Matucci-Cerinic

  2. Division of Medicine, University College London, Centre for Rheumatology, Royal Free Hospital, London, UK

    Christopher P. Denton

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Chaigne, B., Guillevin, L. (2019). Differential Diagnosis: Vasculitis, Rheumatoid Arthritis, Behçet’s Disease, and Thromboembolism. In: Matucci-Cerinic, M., Denton, C. (eds) Atlas of Ulcers in Systemic Sclerosis. Springer, Cham. https://doi.org/10.1007/978-3-319-98477-3_17

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  • DOI: https://doi.org/10.1007/978-3-319-98477-3_17

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