Nephrogenic fibrosing dermopathy
The skin, particularly of the extremities, becomes thickened and hard, leading to immobility and contracture of the joints and difficulty walking. Treatment is unsatisfactory. Systemic involvement (with fibrosis of skeletal muscle, myocardium, lungs, kidney, and testes) has been demonstrated in some patients, where it has been called nephrogenic systemic fibrosis.
The condition develops over days to weeks; about 5% of cases are fulminant and may result in death. Rapid new onset of fluctuating hypertension has been reported to precede skin changes.
The disorder affects males and females equally and has been reported in patients with a variety of ethnic backgrounds.
L91.8 – Other hypertrophic disorders of the skin
424114000 – Nephrogenic fibrosing dermopathy
- Scleroderma (systemic sclerosis)
- Sclerodermoid porphyria cutanea tarda
- 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.
- Increased glycosaminoglycans seen in diabetes.