Nephrogenic systemic fibrosis associated with liver transplantation, renal failure and gadolinium

A 47-year-old man underwent liver transplantation for cirrhosis secondary to hepatitis C and alcoholism. This was complicated by primary donor liver dysfunction and acute renal failure requiring dialysis. Gadolinium magnetic resonance cholangiopancreatography was performed 2 weeks post transplant, and a second successful liver transplant was performed 1 week later. Shortly after this, the patient developed rapidly progressive erythematous plaques over his abdomen, lower and upper limbs. There was marked oedema and skin induration. Fibrosis severely limited his mobility, leaving him wheelchair-bound. An abdominal plaque biopsy revealed increased dermal mucin and cellularity, with proliferation of spindled fibroblastic cells. Paraprotein was not detected in the serum. Facial sparing, the absence of serum paraprotein and the histopathological findings confirmed the diagnosis of nephrogenic systemic fibrosis. Immunohistochemical stains revealed CD34-positive spindle-shaped cells, and electron microscopy did not detect free gadolinium. Following improvement in renal function and various treatments, his plaques softened, fibrosis slowed and mobility partially improved. Gadolinium magnetic resonance cholangiopancreatography was performed following this improvement. Six weeks later, further progression of nephrogenic systemic fibrosis occurred despite normal renal function.