RESUMO
Nephrogenic systemic fibrosis is an idiopathic, sclerosing condition that occurs only in patients who have impaired renal function. Although its most conspicuous manifestation is in the skin, the condition involves multiple organ systems and is potentially fatal. Its postulated association with gadolinium-based magnetic resonance contrast agents has attracted attention. The distinctive clinical features of this entity include a prodromal systemic inflammatory syndrome followed by a chronic course of fibrosis that has a predilection for the skin and subcutaneous tissues of the extremities. The progressive systemic fibrosing process involves multiple organs and contributes to the morbidity and the increased mortality. Appropriate preventive action, prompt recognition and timely reporting of cases may enable better management of this condition.