Nephrogenic Systemic Fibrosis: Late Skin Manifestations | Dermatology | JAMA Dermatology | JAMA Network
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February 2009

Nephrogenic Systemic Fibrosis: Late Skin Manifestations

Author Affiliations

Author Affiliations: Departments of Dermatology (Drs Bangsgaard and Skov) and Pathology (Dr Rossen), University Hospital of Copenhagen Gentofte, Hellerup, and Department of Nephrology, University Hospital of Copenhagen Herlev, Herlev (Dr Marckmann), Denmark. Dr Marckmann is now with the Department of Nephrology, Odense University Hospital, Odense, Denmark.

Arch Dermatol. 2009;145(2):183-187. doi:10.1001/archdermatol.2008.551

Background  Nephrogenic systemic fibrosis (NSF) is a serious disease that occurs in patients with severe renal disease and is believed to be caused by gadolinium-containing contrast agents. A detailed description of the late skin manifestations of NSF is important to help dermatologists and nephrologists recognize the disease.

Observations  We studied 17 patients with NSF late in the disease. All patients showed epidermal atrophy and hairlessness of the affected regions, primarily the lower legs. Affected areas were symmetrically distributed and hyperpigmented in most cases. Eleven patients showed confluent dermal plaques with thickening and hardening. In contrast, 3 patients presented with wrinkled, redundant skin as seen in cutis laxa. Patients with NSF had significantly poorer scores than control patients on the Daily Life Quality Index (mean [SD], 11. 4 [7.4] vs 1.5 [2. 3]; P < .001).

Conclusions  This descriptive case series of patients with NSF gives a detailed clinical picture of the skin manifestations late in the disease. It demonstrates that the clinical picture in the late stage has a varied presentation and that NSF has a significant effect on the quality of life.