Abstract

LOPEZ DE GOICOECHA, ME  and  ACOSTA, V. Nephrogenic systemic fibrosis: A case report and review of the literature. Rev. argent. dermatol. [online]. 2014, vol.95, n.3, pp.1-9. ISSN 1851-300X.

Nephrogenic systemic fibrosis (NSF) is a rare condition appearing only in patients with severe renal impairment and characterized by widespread tissue fibrosis. Although the pathogenesis of this disorder remains unclear, there is evidence of a strong association between gadolinium-based contrast agents (Ga-CA) exposure and the triggering of this disease. Cellular elements involved in pathogenesis of NSF include bone-marrow-derived collagen producing fibrocytes, myofibroblasts and activated macrophages. We present the case of a caucasian female with advanced renal failure who underwent several times magnetic resonance imaging (MRI) procedures using Ga-CA. The patient developed a progressive fibrosing disorder with bilateral indurated papules on the lower legs, joint contractures and hairlessness, and was diagnosed of NSF based on the histologic findings of skin biopsies. Later in the disease she presented with systemic involvement and fatal evolution.

Keywords : Nephrogenic systemic fibrosis; Gadolinium; MRI.