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Case Report/Case Series |

Nephrogenic Systemic Fibrosis Manifesting a Decade After Exposure to Gadolinium

Krista N. Larson, BS1; Amy L. Gagnon, MD1; Melissa D. Darling, MD2; James W. Patterson, MD2; Thomas G. Cropley, MD1
[+] Author Affiliations
1Department of Dermatology, University of Virginia School of Medicine, Charlottesville
2Department of Dermatopathology, University of Virginia School of Medicine, Charlottesville
JAMA Dermatol. 2015;151(10):1117-1120. doi:10.1001/jamadermatol.2015.0976.
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ABSTRACT

Importance  Nephrogenic systemic fibrosis (NSF) is a fibrosing skin disorder that develops in patients with kidney failure and has been linked to exposure to gadolinium-containing contrast agents. The time between exposure to gadolinium and the initial presentation of NSF is typically weeks to months but has been documented to be as long as 3½ years. We report a case of NSF developing 10 years after exposure to gadolinium.

Observations  A long-term hemodialysis patient was exposed to gadolinium several times between 1998 and 2004 during magnetic resonance angiography of his abdominal vessels and arteriovenous fistula. In 2014, he was seen at our clinic with new dermal papules and plaques. Biopsy of affected skin showed thickening of collagen, CD34+ spindle cells, and increased mucin in the dermis, supporting the diagnosis of NSF.

Conclusions and Relevance  The clinical history and histopathological features of this case support the diagnosis of NSF 10 years after exposure to gadolinium. Although the use of gadolinium contrast agents in patients with kidney failure has markedly decreased, patients with exposure to gadolinium years to decades previously may manifest the disease.

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Figure 1.
Patient With Nephrogenic Systemic Fibrosis

A, Dermal papules in linear arrays on the left arm. B, Two indurated dermal plaques in the vicinity of the arteriovenous fistula (arrowheads) on the right volar forearm.

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Figure 2.
Sections Stained With Hematoxylin-Eosin

A, Thickened dermal collagen containing spindle cells and interstitial mucin deposition. B, Magnification of thickened collagen with surrounding clefts containing spindle cells.

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Figure 3.
CD34 Immunohistochemical Staining and Colloidal IronStained Sections

A, CD34 immunohistochemical staining that highlights the fibroblast-like spindle cells. B, Colloidal iron staining that reveals increased interstitial mucin deposition.

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