Publications (2)2.48 Total impact
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ABSTRACT: Nephrogenic fibrosing dermopathy (NFD) is a rare clinical entity affecting patients with renal failure, often on chronic dialysis or after transplantation. The patient profile at risk for this debilitating condition is undefined. Lack of awareness of the condition has hampered epidemiologic work in identifying the etiology. We present four chronic hemodialysis (HD) patients who developed this disease. The patients' ages ranged from 26 to 75 years, and they had received HD from between 20 months and 10 years before the diagnosis of NFD. Two patients had a history of renal transplantation. All patients had progressive thickening and woody induration of the skin associated with contractures, leading to difficult ambulation, and permanent disability within weeks of the diagnosis. In one case, the diaphragm, psoas muscle, and pericardium were involved. The latter is likely the first report of pericardial involvement of NFD. In all four patients, the skin findings were restricted to the extremities, sparing the trunk and face. Skin biopsy findings included thickened dermis with particularly thickened collagen bundles, fibroblast proliferation, minimal mucin deposition, and nearly absent inflammation. The pathologic findings were distinct from scleromyxedema and scleroderma. We found no laboratory evidence of autoimmune disease or thyroid dysfunction to account for alternate etiologies. CD34-positive cells were documented in the skin biopsies as well as in the diaphragm, psoas muscle, and pericardial tissue of the concerned case. NFD is a novel fibrosing disorder of progressively debilitating nature which needs further clinical characterization and recognition to guide investigation of its pathogenesis.
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ABSTRACT: 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.
Saint Louis UniversityСент-Луис, Michigan, United States