Nephrogenic systemic fibrosis
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
Differential Diagnosis & Pitfalls
- Scleroderma (systemic sclerosis)
- 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.
Drug Reaction Data