Nephrogenic Fibrosing Dermopathy After Five Days of Hemodialysis

Nephrogenic fibrosing dermopathy (NFD) is a newly-reported illness associated with renal disease; more specifically, with dialysis. Papules, subcutaneous nodules, and joint contractures are present in the skin. Pathologically, it is very similar to scleromyxedema. Increased dermal fibroblasts, thickened collagen bundles with clefting, and increased mucin are seen in the dermis and subcutis. We report a case of a 53-year-old female with nephrogenic fibrosing dermopathy after 5 days of hemodialysis. The patient experienced progressive bound-down, indurated erythematous plaques on her extremities with limited range of motion and pain shortly after hemodialysis. Biopsy showed increased dermal fibroblasts, thickened collagen bundles with clefting, and increased mucin in the derms and subcutis. No paraproteinemia was found. She did not improve when her renal function improved, nor did prednisone improve her condition. This report and others are vital to help elucidate the etiological factors, course, and best treatment options for this disease process.