A Case of Nephrogenic Systemic Fibrosis

Abstract

We report a case of nephrogenic systemic fibrosis. A patient on hemodialysis due to end stage renal failure was found to have skin sclerosis on the extremities and joint contracture after enhanced MRI examination for acoustic neuroma and blood flow abnormality. The patient was diagnosed with nephrogenic systemic fibrosis based on the absence of ANA and fibrosis of dermis and subcutis and treated with PUVA; however, only slight improvement of skin sclerosis and joint contracture was observed. Ever since Cowper and coworkers first described this new entity, many case reports of this disease have been published from Europe and U. S. that suggest that gadolinium applications for patients with end-stage renal disease might be responsible for this problem. Although some radiologists have recognized that a gadolinium-contrast agent poses a risk for this disease, very few cases have been reported by dermatologists in Japan. Dermatologists should be aware of the etiology and symptoms of nephrogenic systemic fibrosis and recognize that the gadolinium-contrast agent is harmful to hemodialysis patients.