Abstract
Clinically, systemic sclerosis is dominated by two features: small vessel vasculopathy and organ fibrosis. These two pathological features are responsible for the clinical manifestations of Raynaud’s phenomenon, digital ulcers, renal crisis, pulmonary arterial hypertension, skin thickening, interstitial lung disease, cardiac complications, and other organ involvement. In spite of this commonality, there is a great deal of heterogeneity in disease expression in scleroderma with some individuals having mild involvement and others with severe and progressive disease.
This chapter will focus on the more visually apparent complications of SSc including Raynaud’s phenomenon, telangiectasias, pigment changes, skin thickening, contractures, arthritis, and periarthritis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Brand FN, Larson MG, Kannel WB, et al. The occurrence of Raynaud’s phenomenon in a general population: the Framingham Study. Vasc Med. 1997;2:296–301.
Matucci-Cerinic M, Kahaleh B, Wigley FM. Review: evidence that systemic sclerosis is a vascular disease. Arthritis Rheum. 2013;65(8):1953–62.
Walker JG, Stirling J, Beroukas D, Dharmapatni K, Haynes DR, Smith MD, Ahern MJ, Roberts-Thomson PJ. Histopathological and ultrastructural features of dermal telangiectasias in systemic sclerosis. Pathology. 2005;37(3):220–5.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5.
Barnett AJ, Miller MH, Littlejohn GO. A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol. 1988;15:276–83.
Kahaleh MB, Sultany GL, Smith EA, Huffstutter JE, Loadholt CB, LeRoy EC. A modified scleroderma skin scoring method. Clin Exp Rheumatol. 1986;4(4):367–9.
Furst DE, Clements PJ, Steen VD, Medsger Jr TA, Masi AT, D’Angelo WA, Lachenbruch PA, Grau RG, Seibold JR. The modified Rodnan skin score is an accurate reflection of skin biopsy thickness in systemic sclerosis. J Rheumatol. 1998;25(1):84–8.
Medsger Jr TA, Steen VD. Classification, prognosis. In: Clements PJ, Furst DE, editors. Systemic sclerosis. Baltimore, MD: Williams & Wilkins; 1996. p. 51–64.
Poole JL. Musculoskeletal rehabilitation in the person with scleroderma. Curr Opin Rheumatol. 2010;22(2):205–12.
Elhai M, Guerini H, Bazeli R, Avouac J, Freire V, Drapé JL, Kahan A, Allanore Y. Ultrasonographic hand features in systemic sclerosis and correlates with clinical, biologic, and radiographic findings. Arthritis Care Res (Hoboken). 2012;64(8):1244–9.
Walker KM, Pope J, Participating members of the Scleroderma Clinical Trials Consortium (SCTC), Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails—a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012;42(1):42–55.
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
Pope, J., Mayes, M.D. (2014). Skin Manifestations and Musculoskeletal Disease in SSc. 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_3
Download citation
DOI: https://doi.org/10.1007/978-1-4939-0980-3_3
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)