Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is an emerging systemic fibrosing disorder that develops in the setting of renal insufficiency. Nephrogenic fibrosing dermopathy (NFD) is a fibrosing condition of the skin which shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It is charact...

BACKGROUND AND OBJECTIVES Recent studies strongly link nephrogenic systemic fibrosis to gadolinium administration for magnetic resonance imaging. In a recent advisory, the Food and Drug Administration stated that all gadolinium-containing chelates are potentially associated with nephrogenic systemic fibrosis; however, most reported cases are linked to gadodiamide (Omniscan) and gadopentetate di...

Osseous metaplasia has recently been described in several cases of nephrogenic systemic fibrosis, sometimes in association with unusual clinical features such as painful hyperkeratotic spicules, palpable bony masses, and disease regression. Some authors have suggested that it may mainly occur late in the disease course or even be a marker for involuting nephrogenic systemic fibrosis. Here, we p...

Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadolinium-based contrast agents used in magnetic resonance imaging. Because of this relationship between nephrogenic systemic fibrosis and gadolinium-based contrast agents, the U.S. Food and Drug Administration currently warns against u...

We describe a 9-year-old boy on chronic hemodialysis who presented with hyperpigmented, tightly bound-down, indurated plaques of his bilateral lower extremities. Pertinent history included a recent series of magnetic resonance imaging tests utilizing gadolinium-based contrast media. Histopathology showed widened septae with increased fibroblasts and collagen in the subcutis consistent with neph...

BACKGROUND Nephrogenic systemic fibrosis is a debilitating and painful disorder with an increased stimulation of the connective tissue in the skin and systemic tissues. The disease is associated with exposure to gadolinium-based contrast agent used in magnetic resonance imaging in patients with renal impairment. METHODS The prevalence of nephrogenic systemic fibrosis has so far never been det...

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a rare complication of exposure to gadolinium-based contrast agents in patients who have significantly decreased renal function. Manifestations include fibrosis of the skin and other tissues. Effective therapies are lacking. Photopheresis has been tried with variable rates of improvement, and small num...

Nephrogenic systemic fibrosis is a disabling progressive condition that is being reported with increased frequency in patients with kidney disease. Treatment is extremely limited and largely supportive. We report a case of severe nephrogenic systemic fibrosis in a dialysis patient exposed to multiple doses of gadolinium who improved clinically and histologically with treatment with imatinib.