Abstract

Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy (NSF/NFD) is a rare fibrosing disorder that occurs in patients with renal failure. It is associated with significant mortality and morbidity. Patients typically present with painful or pruritic indurated plaques involving the limbs and trunk, with sparing of the face. Severity and rapidity of cutaneous progression correlate with poorer prognosis. To date, the management of NSF/NFD remains anecdotal. The aetiological link in NSF/NFD is also yet to be confirmed, but renal dysfunction seems a common feature. Following recent reports of a possible causative role of gadolinium, we present two patients with histologically confirmed NSF/NFD, who had exposure to gadolinium-containing contrast agents 1-2 months before onset of disease. Severity of renal impairment, lack of immediate dialysis after exposure and cumulative dose of gadolinium are possible factors influencing the development of NSF/NFD. The process of transmetallation of gadolinium chelates may occur in patients with renal impairment, leading to precipitation of free gadolinium in the dermis or other organs, causing tissue injury that ultimately leads to the clinical manifestations of NSF/NFD. Although the causative role is not proven, gadolinium-containing contrast agents should be used only if clearly necessary in patients with renal failure.