A variant of nephrogenic fibrosing dermopathy with osteoclast‐like giant cells: a syndrome of dysregulated matrix remodeling?

Hershko, Klilah; Hull, Cheryl; Ettefagh, Leila; Nedorost, Susan T.; Dyson, Senait W.; Horn, Thomas; Gilliam, Anita C.

doi:10.1111/j.0303-6987.2004.00177.x

Cited by 43 publications

(28 citation statements)

References 11 publications

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“…Cutaneous and muscle calcification has been described in other patients with NFD. 6,7,18 Similar dystrophic calcification is seen in calciphylaxis, a poorly understood vasculopathy that primarily affects patients with renal failure. In this condition, elevated serum calcium or phosphorus levels are believed to play a role in the vascular damage that ultimately leads to infarction of skin and subcutaneous fat.…”

Section: ™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™3mentioning

confidence: 91%

Involvement of skeletal muscle in dialysis‐associated systemic fibrosis (nephrogenic fibrosing dermopathy)

et al. 2004

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Nephrogenic fibrosing dermopathy (NFD), a newly recognized scleroderma-like disease, was originally described as a purely cutaneous disorder. More widespread involvement, including fibrosis of pulmonary and cardiac tissues, has been documented only recently, and it has been suggested that a more appropriate designation is dialysis-associated systemic fibrosis. We report five cases of this novel disorder, spanning a spectrum of primarily skin to primarily muscle involvement. Clinical, radiological, electrophysiological, and pathological studies revealed moderate to severe fibrosis of striated muscles. All patients had end-stage renal failure on chronic dialysis, subacute to chronic hardening of the skin and muscles, restriction of limb movements with joint contractures, but normal to only mildly weak muscle strength. Limitation of movements was caused predominantly by skin tightness and induration, and by joint contractures rather than muscle weakness. Computerized tomography showed fibrosis of the fascia and muscles in the most severely affected patients, and electromyography showed mild to severe myopathic changes. Histopathology of affected muscles revealed a spectrum of mild to severe fibrosis, degenerating fibers, and chronic inflammatory cells. These results further support the contention that NFD is not a purely cutaneous disease, but is part of a larger systemic fibrotic process that may involve muscles.

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confidence: 91%

Involvement of skeletal muscle in dialysis‐associated systemic fibrosis (nephrogenic fibrosing dermopathy)

et al. 2004

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“…Osseous metaplasia, osteoclastlike giant cells, and calciphylaxis can be seen in some NSF biopsies. [11][12][13] On a technetium 99 -diphosphosphonate scan, areas of increased uptake can be observed in muscles. 14,15 On magnetic resonance imaging, axial T1-and fat-suppressed T2-weighted images show symmetric skin thickening and soft tissue edema.…”

Section: Introductionmentioning

confidence: 99%

New Insights into Nephrogenic Systemic Fibrosis

Swaminathan

Shah

2007

Journal of the American Society of Nephrology

111

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“…6 Cases with calcification, osteoclast-like giant cells, and osseous metaplasia have also been reported. 7,8 A patient with a history of renal failure presented to our clinic with a localized linear plaque resembling a hypertrophic scar, along a previously traumatized blood vessel on the left forearm. Histologic examination revealed classic changes of NFD: a proliferation of CD34 1 fibrocytes between delicate collagen bundles and interstitial dermal mucin.…”

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confidence: 99%