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Nephrogenic systemic fibrosis
Other Resources UpToDate PubMed

Nephrogenic systemic fibrosis

Contributors: Christine S. Ahn MD, FAAD, William W. Huang MD, MPH, FAAD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy (NFD), is an uncommon disorder without any sex or race predilection. It is associated with exposure to gadolinium-containing contrast agents during magnetic resonance imaging (MRI) studies in patients with advanced renal disease. Other possible risk factors include severity of renal failure, thrombotic episodes, edema, vascular procedures, and erythropoietin exposure. The number of cases per year has significantly decreased after the recognition of the causal link between gadolinium-based contrast exposure and renal dysfunction.

NSF presents with thick, hard plaques on the extremities and trunk, leading to immobility and contracture of the joints. Systemic involvement (with fibrosis of skeletal muscle, myocardium, lungs, kidney, and testes) has been demonstrated in some patients.

The condition develops over days to weeks. About 5% of cases are fulminant and may result in death. The rapid new onset of fluctuating hypertension has been reported to precede skin changes.

Codes

ICD10CM:
L90.8 – Other atrophic disorders of skin

SNOMEDCT:
424114000 – Nephrogenic systemic fibrosis

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Last Reviewed:06/24/2018
Last Updated:09/30/2019
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Nephrogenic systemic fibrosis
A medical illustration showing key findings of Nephrogenic systemic fibrosis : Bone pain, Edema, Joint contractures, Symmetric extremities distribution, Thickened skin, Pruritus, Hardened skin, Feet, Hands, Hyperpigmentation, Smooth plaques
Clinical image of Nephrogenic systemic fibrosis - imageId=3417564. Click to open in gallery.  caption: 'A swollen hand with unrelated crusting and erythema.'
A swollen hand with unrelated crusting and erythema.
Copyright © 2024 VisualDx®. All rights reserved.