Nephrogenic systemic fibrosis
Alerts and Notices
Synopsis
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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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Scleroderma (systemic sclerosis)
- Morphea
- Sclerodermoid porphyria cutanea tarda
- Sclerodermoid 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.
- Spanish toxic oil syndrome – Associated with ingestion of contaminated cooking oil.
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Management Pearls
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Therapy
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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.Subscription Required
References
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Last Reviewed:06/24/2018
Last Updated:09/30/2019
Last Updated:09/30/2019
Nephrogenic systemic fibrosis