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A Report on 4 New Cases of Nephrogenic Fibrosing Dermopathy in Chronic Hemodialysis Patients

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Nephrogenic fibrosing dermopathy (NFD) is a rare entity affecting patients with renal failure, often on chronic dialysis or after transplantation (TXP). The patient profile at risk for this debilitating condition is yet undefined as is the role of renal failure in its etiology. We diagnosed 4 chronic hemodialysis (HD) patients with NFD. A 55-year-old Caucasian male on HD for 5 years, secondary to diabetic nephropathy, developed woody, indurated skin of the extremities, decreased mobility, and wheelchair dependence. He died within 1 year. A 66-year-old African-American male with diabetes, hypertension, and pancreatic cancer developed thick indurated skin on his extremities after being on HD for approximately 20 months. He died 3 months later from sepsis. A 26-year-old Caucasian female on HD for approximately 10 years, secondary to hyperoxaluria-induced renal failure, had undergone combined liver and kidney TXP with primary nonfunction of the renal TXP. She succumbed to cholangitis approximately 1 year after progressive skin thickening and joint contractures were noted. A 75-year-old Caucasian female with renal failure secondary to recurrent hemolytic uremic syndrome and TXP failure was dialysis dependent for 6 years. Over a 3-month period, she developed skin changes consistent with NFD and entered hospice care secondary to marked deterioration in her quality of life. In all of these cases, skin changes were restricted to the extremities, sparing the trunk, face, and internal organs. Skin biopsy findings included thickened dermis with particularly thickened collagen bundles, mucin deposition, and fibroblast proliferation and were distinct from scleromyxedema and scleroderma. Autoimmune disease workup was negative. Indeed, NFD is a novel cutaneous fibrosing disorder of progressively debilitating nature in patients with renal failure that needs further clinical and pathological characterization.

Document Type: Research Article


Affiliations: 1: St. Louis University Division of Nephrology 2: Washington University Division of Rheumatology, St. Louis, MO, U.S.A.

Publication date: January 1, 2004


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