A follow-up of four cases of nephrogenic systemic fibrosis: is gadolinium the specific trigger?

We originally reported four patients with nephrogenic systemic fibrosis (NSF) in the British Journal of Dermatology in 2003, and now present an update of their outcome. All four patients (three females and one male) presented within a six month period at our institution, and had received gadolinium contrast agents prior to disease onset. Two patients with functioning renal transplants had limited cutaneous disease, two patients maintained on haemodialysis had more severe disease and died one year after disease onset. Gadolinium deposition was demonstrated in lesional skin of one patient using electron microscopy and energy dispersive spectroscopy.