ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (8)
Nephrogenic systemic fibrosis
Print
Other Resources UpToDate PubMed

Nephrogenic systemic fibrosis

Print Images (8)
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 gender 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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required

References

Subscription Required

Last Reviewed: 06/25/2018
Last Updated: 07/12/2018
Copyright © 2019 VisualDx®. All rights reserved.
Nephrogenic systemic fibrosis
Print 8 Images
View all Images (8)
(with subscription)
Nephrogenic systemic fibrosis : Bone pain, Edema, Joint contractures, Symmetric extremities, Thickened skin, Pruritus, Hardened skin, Feet, Hands, Hyperpigmentation, Smooth plaques
Clinical image of Nephrogenic systemic fibrosis
Copyright © 2019 VisualDx®. All rights reserved.