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Digital ulcers (DUs) occurring on the fingers in patients with systemic sclerosis (SSc) are associated with substantial pain and disability and are often challenging to treat. However, careful clinical assessment and prompt intervention (wound bed management and systemic pharmacologic treatment) may modify the clinical course.
To provide a practical approach to the assessment and management of SSc-DUs and highlight unmet needs and research priorities.
A narrative review of the extant literature was undertaken to provide a broad overview of current knowledge and augmented by expert opinion.
Half of the patients with SSc have a history of DUs, and there is a point of prevalence of approximately 10%. Digital ulcers are often very painful and affect all aspects of physical, social, and family life as well as occupation. Digital ulcers are associated with a severe disease course. Systemic sclerosis DUs, particularly those occurring on the fingertips, represent a vascular ischemic complication, although other etiopathogenic factors play an important role. To guide management, a structured clinical approach is required, including DU definition, classification, and categorization. Digital ulcers require a multidisciplinary approach with close cooperation between physicians and specialist nursing and other allied health professionals to guarantee the appropriate treatment and provide patient education. Local wound bed management is necessary for all DUs and is combined with systemic (pharmacologic) treatments. When treating a DU, the clinician should actively review the therapeutic strategy to prevent further DUs, including the level of systemic disease control, and monitor closely for the development of DU complications, including infection and progression to gangrene. Despite a wide available therapeutic armory, a number of unmet needs and challenges remain that that require resolution to optimize DU management.
Conclusions and Relevance
A practical approach to DU management, including local wound bed management and systemic treatments, is useful. Digital ulcers are of interest to a broad range of dermatologists, rheumatologists, and other physicians providing care for patients with SSc. Careful clinical assessment and prompt intervention can substantially modify the clinical course of DUs in SSc.
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Accepted for Publication: October 27, 2020.
Published Online: May 26, 2021. doi:10.1001/jamadermatol.2021.1463
Corresponding Author: Michael Hughes, PhD, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, S10 2JF, United Kingdom (email@example.com).
Author Contributions: Dr Hughes had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Concept and design: Hughes, El Aoufy, Denton, Khanna, Krieg, Matucci-Cerinic.
Acquisition, analysis, or interpretation of data: Hughes, Allanore.
Drafting of the manuscript: Hughes, Denton, Matucci-Cerinic.
Critical revision of the manuscript for important intellectual content: All authors.
Administrative, technical, or material support: Hughes.
Supervision: Allanore, Khanna, Krieg, Matucci-Cerinic.
Conflict of Interest Disclosures: Dr Hughes has received speaking fees from Actelion. Dr Allanore has had consultancy relationships and/or has received research funding from Actelion, Bayer, Boehringer Ingelheim, Genentech/Roche, Inventiva, Medsenic, and Sanofi in the area of potential treatments of scleroderma and its complications. Dr Denton has received grants from Servier; grants and personal fees from GSK, Arxx Therapeutics, Roche, and CSL Behring; and personal fees from Boehringer Ingelheim, Galapagos, and Horizon. Dr Khanna has received grants from National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases during the conduct of the study and grants from Immune Tolerance Network, BMS, and Pfizer; grants and personal fees from Bayer, Horizon, Boehringer Ingelheim, and Galapagos; personal fees from Acceleron, Actelion, Amgen, AstraZeneca, Blade Therapeutics, CSL Behring, Corbus, Cytori, Genentech/Roche, GSK, Merck, Mitsubishi Tanabe Pharma, Regeneron, Sanofi-Aventis, GlaxoSmithKline, and United Therapeutics; other support from Eicos Sciences Inc; and other support from CiviBioPharma/Eicos Sciences Inc outside the submitted work. Dr Krieg has received speaking fees from Actelion. Dr Matucci-Cerinic has received consulting fees or honorarium from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, and Roche. No other disclosures were reported.
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