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Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy in advanced renal failure

Dana Miskulin, MD
Michael R Rudnick, MD
Section Editor
Thomas A Golper, MD
Deputy Editor
Alice M Sheridan, MD


Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder seen only in patients with kidney failure. It is characterized by two primary features [1,2]:

Thickening and hardening of the skin overlying the extremities and trunk

Marked expansion and fibrosis of the dermis in association with CD34-positive fibrocytes

NSF was originally named nephrogenic fibrosing dermopathy (NFD) because of the characteristic skin findings. However, subsequent studies showed that some patients had fibrosis of deeper structures, including muscle, fascia, lungs, and heart [1]. Because of the systemic findings, nephrogenic systemic fibrosis is preferred to nephrogenic fibrosing dermopathy [3]. (See 'Systemic involvement' below.)

NSF has also been called dialysis-associated systemic fibrosis [4,5]. However, this term is too narrow since the disease is not limited to patients on dialysis [3].

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Literature review current through: Nov 2017. | This topic last updated: Jul 18, 2016.
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