Description of 12 Cases of Nephrogenic Fibrosing Dermopathy and Review of the Literature
Section snippets
Methods
Clinical, laboratory, and histopathological studies were performed in 10 patients evaluated at the Scleroderma Center of Thomas Jefferson University between the years 2000 and 2005, in 1 patient followed at the Veterans Administration Hospital of St. Louis, MO, and in 1 patient followed at the Robert Wood Johnson Medical School (Camden, NJ). This evaluation included a detailed assessment for the presence of systemic involvement, including a review of systems and physical examination for
Demographical and Epidemiological Characteristics
The details of the epidemiologic and demographic data of the 12 patients we studied are shown in Table 1. Ten patients received hemodialysis (HD), 1 patient received peritoneal dialysis (PD), and another patient was on HD at the time of diagnosis but had received PD previously. Six of 12 patients had renal transplantation which failed in all and required removal. The cause of the renal failure was diverse, as well as the duration of dialysis (Table 1).
Clinical Features
The clinical features of the 12 patients we
Discussion
NFD is a severe, usually progressive, fibrotic disorder occurring in patients with end-stage renal disease who received, with few exceptions, hemodialysis or peritoneal dialysis. In addition to the severe cutaneous alterations, frequently there is evidence of systemic involvement including: (1) a very marked elevation of the erythrocyte sedimentation rate and C-reactive protein; (2) tendinous, peri-articular, and striated muscle involvement; and (3) visceral fibrosis of heart, lungs, and other
Acknowledgments
This work was supported by NIH Grant AR19161 (to S.A.J.). Dr. Nora Sandorfi was supported by NIH training Grant AR07583. Dr. Sonsoles Piera-Velazquez was supported by a Postdoctoral Fellowship from the Arthritis Foundation. The expert assistance of Kate Salmon in the preparation of this paper is gratefully acknowledged.
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Supported by NIH Grant AR19616.