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Case Studies in Systemic Sclerosis pp 85–94Cite as

A 65-Year-Old Woman with Hardened Skin, Joint Contractures, and Yellow Scleral Plaques

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Abstract

Nephrogenic systemic fibrosis is a debilitating disease that affects patients with chronic kidney disease after exposure to gadolinium-containing contrast agents. Patients typically exhibit hyperpigmentation, marked induration, and tethering of skin on their extremities with fixed flexion contractures of the fingers, elbows, and knees. Yellow scleral plaques with capillary dilatation may be evident on the eyes, and visceral involvement can cause significant morbidity and mortality. Skin biopsy demonstrates increased numbers of spindle-shaped fibroblast-like cells that are CD34+/procollagen I+  in the dermis and thickened collagen bundles separated by adjacent clefts, and serves to confirm the diagnosis of nephrogenic systemic fibrosis in a patient with characteristic clinical features. Treatment with tyrosine kinase inhibitors may reverse existing fibrosis and prevent progression of disease, although controlled clinical trials are lacking, in part, due to the relative rarity of this condition.

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Author information

Authors and Affiliations

  1. Rheumatology Division, Department of Medicine, UMass Memorial Medical Center, University of Massachusetts Medical School, 119 Belmont Street, Worcester, MA, 01605, USA

    Jonathan Kay

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  1. Jonathan Kay
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Editors and Affiliations

  1. , Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, 29403, USA

    Richard M. Silver

  2. Royal Free and University College, Medical School, University College London, Rowland Street, London, NW3 2PF, United Kingdom

    Christopher P. Denton

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© 2011 Springer-Verlag London Limited

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Kay, J. (2011). A 65-Year-Old Woman with Hardened Skin, Joint Contractures, and Yellow Scleral Plaques. In: Silver, R., Denton, C. (eds) Case Studies in Systemic Sclerosis. Springer, London. https://doi.org/10.1007/978-0-85729-641-2_9

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  • DOI: https://doi.org/10.1007/978-0-85729-641-2_9

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  • Publisher Name: Springer, London

  • Print ISBN: 978-0-85729-640-5

  • Online ISBN: 978-0-85729-641-2

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