What is nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy)?

Nephrogenic systemic fibrosis is a recently described systemic disorder with prominent cutaneous findings seen in patients with impaired renal function who have received gadolinium-based contrast media. Many, but not all, of the patients have been on hemodialysis. It presents as thickened or edematous skin that primarily affects the extremities and trunk. Some patients may also demonstrate discrete papules or plaques. In severe cases, there may be restriction of movement or disabling contracture of the joints. Fibrosis may also involve extracutaneous sites including the sclera (yellow scleral plaques), the heart, lungs, and the skeletal muscle. Histologically, biopsies demonstrate increased numbers of CD34- positive and procollagen-positive fibroblasts and increased mucin in early lesions with marked fibrosis in later lesions, often involving the subcutaneous fatty septae. By energy dispersive spectroscopy, particles of gadolinium can be detected within involved tissues. Treatment has been disappointing as the condition is often refractory to all treatments.

High WA, Ayers RA, Chandler J, et al: Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis, J Am Acad Dermatol 56:21–26, 2007.