NEPHROGENIC SYSTEMIC FIBROSIS

By Gary M. White, MD

nephrogenic systemic fibrosis


Indurated skin lesions in patients exposed to gadolinium was initially termed nephrogenic fibrosing dermopathy. Because many other bodily systems may be affected, the term was changed to nephrogenic systemic fibrosis (NSF). A typical history is exposure to gadolinium-containing contrast agents used for MRI in patients with severe renal disease.

Clinical

Diffuse induration of the skin and flexion contractures occur. Onset is usually months to a few years after exposure, but one case of NSF developing a decade after has been reported [JAMA Derm 2015;151;1117]. Gadolinium-Associated Plaques are erythematous plaques 0.5-2.5 cm in diameter in a patient given gadolinium and are associated with NSF.

Treatment

Unfortunately, NSF has a high mortality rate. To date, there is no proven effective therapy, other than renal transplantation or recovery of the acute kidney injury [JAAD 2016;74;247]. Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF [JAAD 2017;235].

Additional Pictures

The patient is unable to extend the fingers due to the indurated skin.
nephrogenic systemic fibrosis

A little hard to photograph, but the skin of the forearm was very thick and indurated.
nephrogenic systemic fibrosis

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