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Nephrogenic systemic fibrosis (NSF) is a recently described, debilitating systemic disease most commonly seen in patients with renal insufficiency. Exposure to gadolinium-containing contrast agent has been associated with the onset of symptoms. The epidemiology, pathogenesis, clinical manifestation, diagnosis, histolopathology, differential diagnosis and treatment will be reviewed. Clinicians should have a high index of suspicion in susceptible individuals.

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