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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


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.


L90.8 – Other atrophic disorders of skin

424114000 – Nephrogenic systemic fibrosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Scleroderma (systemic sclerosis)
  • Morphea
  • Sclerodermoid Porphyria cutanea tarda
  • Sclerodermoid Acute graft-versus-host disease
  • Scleromyxedema – Tends to have head / neck / upper trunk involvement and biopsy differs.
  • Eosinophilic fasciitis – Biopsy differs, eosinophilia, hypergammaglobulinemia.
  • Pretibial myxedema – Abnormal thyroid function tests.
  • Eosinophilia-myalgia syndrome – Associated with ingestion of contaminated L-tryptophan.
  • Toxic oil syndrome – Associated with ingestion of contaminated cooking oil.

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Management Pearls

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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.

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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.