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Journal of the American Academy of Dermatology
Volume 58, n° 2
pages 336-339 (février 2008)
Doi : 10.1016/j.jaad.2007.10.491
Case Reports

Localized nephrogenic fibrosing dermopathy: Aberrant dermal repairing?

April C. Deng, MD, PhD , Donna Martin Bilu, MD, Bahram Sina, MD, Anthony Gaspari, MD
Department of Dermatology, University of Maryland, School of Medicine, Baltimore, Maryland 

Reprint requests: April C. Deng, MD, PhD, Department of Dermatology, University of Maryland, School of Medicine, 405 West Redwood St, 6th Floor, Baltimore, MD 21201.

Nephrogenic fibrosing dermopathy (NFD) has emerged as a clinicopathologic entity since 2000 and was recently renamed nephrogenic systemic fibrosis. The cause and pathogenesis remain uncertain. The classic clinical presentation is diffuse thickening and hardening of the skin that occurs in patients with renal insufficiency, with or without systemic involvement. We report a patient with renal failure who presented to our dermatology clinic with a localized plaque on the left forearm along the vein that was traumatized during the infusion of erythropoietin. Histologic examination revealed a dermal proliferation of CD34+ fibrocytes with collagen fibers and interstitial mucin accumulation, features characteristic for NFD. We conclude that NFD may present as a localized, scarlike plaque after trauma and exhibit overlapping histopathologic features resembling cicatrix and other dermal reparative/regenerative processes. NFD may, in fact, be a disorder of aberrant extracellular matrix remodeling in patients with renal insufficiency. This is a single case observation with discussion of literature and attempted hypothesis on pathogenesis. No experimental evidence is provided.

The full text of this article is available in PDF format.

 Funding sources: None.
 Conflicts of interest: None declared.

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