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Mayo Clinic specialists are experienced in treating nephrogenic fibrosing dermopathy (NFD), a severe skin disorder that occurs in patients with kidney diseases. Typically, a kidney specialist (nephrologist) and a dermatologist work together to treat the disease. Sometimes a physical medicine and rehabilitation specialist is involved. Mayo physicians are continually exploring new treatment methods.
Skin biopsies are used to diagnose this condition. The cause of NFD has not been clearly determined. NFD is almost always associated with kidney failure, but it can rarely overlap with similar skin conditions that occur with calcium abnormalities, hepatitis C, lupus, blood clots and other conditions. Diagnosis during its early stages may help slow the disease's progression. Read more about nephrogenic fibrosing dermopathy diagnosis.
There is no cure for this disease, nor is there a consistently successful treatment. Some medications can help some patients, but many have serious side effects. Mayo physicians have determined that, in some patients, reducing high doses in erythropoietin therapy might help. Read more about nephrogenic fibrosing dermopathy treatment options.
Nephrogenic fibrosing dermopathy (NFD) is an uncommon disease that seems to occur exclusively in patients with kidney disease. NFD may resemble skin diseases such as scleroderma and scleromyxedema with thickening, masses and excess pigmentation developing on large areas of the skin. The disease can also involve internal organs and can cause permanent shortening of muscles and tendons or scar tissue, thus limiting the range of motion of patients. Most patients have some evidence of a systemic inflammatory response. Growing evidence of involvement of other areas besides the skin has led to increasing use of the term nephrogenic systemic fibrosis to describe the disease.
NFD can start and progress quite quickly, and then stop within a few months. But in that time it can cause severe disability, even death. The disease occurs in patients of all ages, but usually strikes the middle-aged, most often patients who have undergone dialysis for renal failure. It has been reported in some kidney transplant patients and some patients with disorders that result in poorly functioning kidneys
A study by the Centers for Disease Control (CDC) identified exposure to gadolinium-containing contrast agents during magnetic resonance imaging (MRI) studies as a significant risk factor for development of this disease among patients with moderate to end-stage kidney disease. The CDC recommends against using this contrast agent in patients with advanced renal failure. See CDC report.
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