Nephrogenic Fibrosing Dermopathy

Nephrogenic fibrosing dermopathy (NFD) is an acquired, idiopathic, chronic, progressive eruption of the skin sometimes accompanied by systemic fibrosis that occurs in the context of renal failure. The age of patients with NFD ranges from 8 to 87 years at the time of onset, with a mean age of 46.4 years. Most cases have been reported in the US and Europe, but recently cases have also been reported in India. About 175 cases have been noted in a registry established for NFD patients. Even in the setting of renal disease, NFD still occurs rarely and its cause is unknown. Patients present with hard, indurated, sometimes peau d’orange plaques. They often show a distinctive physical appearance, with elbows and knees angled inward, and most patients complain of a loss of range of motion. Histology demonstrates thickened collagen bundles with surrounding clefts, mucin, and a proliferation of fibroblasts and elastic fibers, sometimes with reticular, dermal, large epithelioid or stellate spindle cells. Fibrosis of NFD can affect the internal organs and thus the term nephrogenic systemic fibrosis has been used by some analysts to describe this fibrosing process. The fibrosis of NFD appears to be due to the effects of a cell referred to as the circulating fibrocyte. Rare cases of partial-to-complete spontaneous resolution have been reported in the absence of specific therapy. Treatments yield inconsistent results and include restoration of renal function, extracorporeal photopheresis, photodynamic therapy, high-dose intravenous immunoglobulin, and other immunosuppressive therapies.