Nephrogenic fibrosing dermopathy

Markus, Jodi; James, Alaina J.; Nunez-Gussman, Janna; Sheehan, Andrea M.; Fegan, Laura; Hsu, Sylvia

doi:10.1016/j.jaad.2004.06.019

Cited by 8 publications

(3 citation statements)

References 3 publications

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“…8 Swartz et al 9 noted focal dermal calcification in 1 of 13 NSF patients, but further details were not published. Markus et al 10 reported subcutaneous stromal calcification in two of four NSF patients. Finally, Lewis et al 11 reported one case with clinically and histologically overlapping NSF and calciphylaxis.…”

Section: Discussionmentioning

confidence: 98%

Nephrogenic systemic fibrosis associated with stromal and vascular calcification, report of two cases

Song

Volkov²,

Shea

et al. 2009

J Cutan Pathol

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Nephrogenic systemic fibrosis (NSF) is a systemic fibrosing disorder characterized by the development of large indurated plaques on the skin, primarily in patients with end-stage renal disease (ESRD). Skin biopsy reveals an increased frequency of CD34(+) and Factor XIIa+ cells. Microscopic calcification has been reported in the skin biopsies of rare cases of NSF. The etiology and significance of this calcification is not clear. We present two cases of typical NSF, for which marked stromal and vascular calcification was identified on skin biopsy. In both cases, the calcification was within typical NSF lesions and was intimately associated with aggregates of CD34(+), Factor XIIIa+ spindle and stellate cells. This particular pattern of calcification strongly argues against either nonspecific dystrophic calcification or coincidental metastatic calcification. These findings suggest that calcification may be intrinsic to the pathophysiology of NSF, at least in a subset of NSF patients. In addition, the vascular calcification involving small arteries and arterioles in these two cases morphologically resembled calciphylaxis, which is another poorly understood complication of ESRD. This resemblance raises the possibility that NSF may predispose to or develop in the vicinity of indolent lesions of early-stage calciphylaxis. We propose that skin biopsies performed as part of a diagnostic workup for suspected NSF should preferably include the deep dermis and subcutis, in order to assess possible vascular calcification.

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Section: Discussionmentioning

confidence: 98%

Nephrogenic systemic fibrosis associated with stromal and vascular calcification, report of two cases

Song

Volkov²,

Shea

et al. 2009

J Cutan Pathol

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“…Recently, it has been reported that a spectrum of abnormal calcifications, such as dystrophic calcinosis cutis, benign nodular calcifications, metastatic calcinosis cutis, and osseous metaplasia, develop in NSF patients [10][11][12][13][14][15][16][17][18]. Although many NSF patients have been diagnosed, there is no consensus on the etiology or significance of abnormal calcification in NSF.…”

Section: Introductionmentioning

confidence: 99%

“…However, many reports suggest that calcification may be intrinsic to the disease process [10], [11][12][13][14][15][16][17][18]. Tissue calcification has long been considered to be a passive, degenerative, and endstage process of atherosclerosis and inflammation, while an increasing number of studies, especially in the field of vascular calcification, have shown that calcification is an actively regulated process similar to osteogenesis [20][21][22][23].…”

Section: Introductionmentioning

confidence: 99%

New insights into the mechanism of abnormal calcification in nephrogenic systemic fibrosis – gadolinium promotes calcium deposition of mesenchymal stem cells and dermal fibroblasts

Okada

Yamanaka

Ishikawa

2011

Journal of Dermatological Science

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Nephrogenic systemic fibrosis in liver disease: A systematic review

Mazhar

Shiehmorteza

Kohl

et al. 2009

Magnetic Resonance Imaging

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Nephrogenic systemic fibrosis (NSF) may develop in patients with liver disease, a fact highlighted by Food and Drug Administration (FDA) announcements cautioning against the use of gadolinium‐based contrast agents (GBCAs) in select liver disease patients. The purpose of this systematic literature review is to characterize the risk of NSF in patients with liver disease. All published articles on NSF from September 2000 through August 2008, were identified via PubMed searches and examination of articles' reference lists. Two reviewers independently read each article and identified unique patients with biopsy‐proven or suspected NSF. Data on demographics, liver status, renal status, and GBCA exposure were collected. A total of 324 articles were reviewed, with 108 articles containing case descriptions of 335 unique NSF patients. After excluding the 95/335 (28%) patients in whom the presence or absence of liver disease was uncertain, liver disease was confirmed present in 41/239 (17%) patients. Renal insufficiency could be assessed in 35 of the liver disease patients; severe renal insufficiency, defined as a glomerular filtration rate (GFR) or estimated GFR (eGFR) <30 mL/min/1.73 m2 or dialysis requirement, was present in 34/35 (97%) patients. The lone patient who developed NSF with mild/moderate renal insufficiency was atypical and received a total gadodiamide load of 0.76 mmol/kg over a 10‐week period periliver transplantation. The published medical literature demonstrates that patients with liver disease who develop NSF also have severe renal insufficiency, suggesting that liver disease does not confer a risk for NSF beyond that of the underlying renal insufficiency. J. Magn. Reson. Imaging 2009;30:1313–1322. © 2009 Wiley‐Liss, Inc.

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Nephrogenic fibrosing dermopathy

Cited by 8 publications

References 3 publications

Nephrogenic systemic fibrosis associated with stromal and vascular calcification, report of two cases

Nephrogenic systemic fibrosis associated with stromal and vascular calcification, report of two cases

New insights into the mechanism of abnormal calcification in nephrogenic systemic fibrosis – gadolinium promotes calcium deposition of mesenchymal stem cells and dermal fibroblasts

Nephrogenic systemic fibrosis in liver disease: A systematic review

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