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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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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