Description of twelve cases of nephrogenic fibrosing dermopathy and review of the literature
Fabian A. Mendoza, Thomas Jefferson University; Carol M. Artlett, Thomas Jefferson University; Nora Sandorfi, Thomas Jefferson University; Kevin Latinis, Kansas University Medical Center; Sonoles Piera-Velazquez, Thomas Jefferson University; and Sergio A. Jimenez, Thomas Jefferson University

DATE: February 2006
SOURCE: Seminars in Arthritis and Rheumatism, vol35, iss4, pp.238-249

263 Mendoza et al (SAR) .doc

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ABOUT THIS DOCUMENT:
This article has been peer reviewed. It is the authors' final version prior to publication in Seminars in Arthritis and Rheumatism Volume 35, Issue 4, February 2006, Pages 238-249. The published version is available at DOI: 10.1016/j.semarthrit.2005.08.002. Copyright © Elsevier.

ABSTRACT:

ABSTRACT

Objectives: To review the clinical and laboratory features of twelve cases of nephrogenic fibrosing dermopathy (NFD) studied at our institution and of 70 previously described cases in the literature.

Methods: Clinical evaluation and laboratory studies of twelve patients with NFD associated with chronic hemodialysis or peritoneal dialysis for end-stage renal disease and a review of 23 previous publications describing 70 patients with this disease.

Results: Eleven patients undergoing chronic hemodialysis and one patient undergoing chronic peritoneal dialysis for end-stage renal failure developed a severe and progressive cutaneous fibrotic process with woody induration of legs, thighs, hands and forearms, and severe loss of motion and flexion contractures in multiple joints. Several patients displayed systemic involvement including fibrosis of muscles, myocardium and lungs and marked elevations of the erythrocyte sedimentation rate and/or C reactive protein. Three patients died within two years of onset of symptoms. A review of previously published reports of this disorder confirmed the presence of systemic involvement and a poor prognosis with a high rate of mortality.

Conclusions: The analysis of twelve cases and a review of 70 previously described cases indicate that NFD is a severe and usually progressive systemic fibrotic disease affecting the dermis, subcutaneous fascia and striated muscles. It also appears that the disease can cause fibrosis of lungs, myocardium and other organs.