Abstract
Systemic sclerosis (scleroderma) is unique among the rheumatic diseases because a widespread obliterative vasculopathy exists involving the peripheral arteries and microcirculation. Various forms of digital ulceration and tissue breakdown are recognized. This chapter illustrates these different lesions by presenting representative examples of each type.
Access provided by Autonomous University of Puebla. Download chapter PDF
Similar content being viewed by others
Keywords
Introduction
Systemic sclerosis (scleroderma) is unique among the rheumatic diseases because a widespread obliterative vasculopathy exists involving the peripheral arteries and microcirculation [1, 2]. Vascular changes involving capillaries, arterioles, and small arteries are well documented. Macrovascular disease in larger peripheral arteries is also common [3]. Pathological specimens from digital vessels demonstrate striking intimal thickening with marked luminal narrowing and evidence of thrombi, while the smooth muscle of the media is usually normal. The involved vessels normally are important in both tissue nutrition and body thermoregulation. Raynaud’s phenomenon is often the initial clinical manifestation of this peripheral vascular disease (Figs. 5.1 and 5.2). The abnormal response to cold and stress is caused by the abnormalities in the regulation of regional blood flow in the skin and digits.
Scleroderma is also associated with fibrotic skin, especially of the fingers. Fibrotic skin changes in association with the peripheral vascular disease lead to skin hypoxia, critical ischemia, and eventual tissue injury. Digital ulcers are reported in about 25–50 % of patients with scleroderma [4]. Prolonged critical ischemia can cause deep tissue injury and digital loss. Digital amputation secondary to occlusion of digital arteries occurs in a subset of (about 11 %) patients, usually with limited skin disease with the presence of anti-centromere antibody [5].
Various forms of digital ulceration and tissue breakdown are recognized (Figs. 5.3, 5.4, 5.5, 5.6, 5.7, 5.8, 5.9, 5.10, 5.11, 5.12, 5.13, 5.14, 5.15, 5.16, 5.17, 5.18, 5.19, 5.20, 5.21, 5.22, 5.23, 5.24, 5.25, and 5.26). These include distal digital ischemic ulcers secondary to digital artery and skin arteriole disease; breakdown of islands of skin on the digits due to microvascular occlusion in fibrotic skin; skin fissures due to drying of skin surface; ulcerations at the site of joint contractures where fragile skin is easily traumatized; deep tissue injury leading to gangrene; and loss of digits due to macrovascular occlusion of major peripheral arteries in the arm, palm, or fingers. Similar events occur in the lower extremities. This chapter illustrates these different lesions by presenting representative examples of each type of lesion.
Treatment of these lesions includes vasodilators, protection from repeated trauma, and attention to good wound care [6–8].
References
Rodnan GP, Myerowitz RL, Justh GO. Morphologic changes in the digital arteries of patients with progressive systemic sclerosis (scleroderma) and Raynaud phenomenon. Medicine. 1980;59:393–408.
Matucci-Cerinic M, Kahaleh B, Wigley FM. Review: evidence that systemic sclerosis is a vascular disease. Arthritis Rheum. 2013;65(8):1953–62.
Veale DJ, Collidge TA, Belch JJ. Increased prevalence of symptomatic macrovascular disease in systemic sclerosis. Ann Rheum Dis. 1995;54(10):853–5.
Steen V, Denton CP, Pope JE, Matucci-Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology (Oxford). 2009;48 Suppl 3:iii19–24.
Denton CP, Krieg T, Guillevin L, Schwierin B, Rosenberg D, Silkey M, Zultak M, Matucci-Cerinic M, DUO Registry Investigators. Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry. Ann Rheum Dis. 2012;71(5):718–21.
Herrick AL. Management of Raynaud’s phenomenon and digital ischemia. Curr Rheumatol Rep. 2013;15(1):303.
Tingey T, Shu J, Smuczek J, Pope J. Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res (Hoboken). 2013;65(9):1460.
Lambova S, Müller-Ladner U. Connective tissue diseases: treatment of digital ulcers in systemic sclerosis. Nat Rev Rheumatol. 2011;7(1):5–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media New York
About this chapter
Cite this chapter
Wigley, F.M. (2014). Ischemic Digital Ulcers. In: Mayes, M. (eds) A Visual Guide to Scleroderma and Approach to Treatment. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0980-3_5
Download citation
DOI: https://doi.org/10.1007/978-1-4939-0980-3_5
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4939-0979-7
Online ISBN: 978-1-4939-0980-3
eBook Packages: MedicineMedicine (R0)