Nephrogenic Fibrosing Dermopathy

Diagnosis

A skin biopsy (deep biopsy) is used to diagnose this condition.

Some signs and symptoms of the disease include:

• Hardened masses on the skin, thickening and hardening of the skin and excess pigmentation. Skin changes may begin with red or dark patches or nodules. The skin may become shiny, feel "woody" and develop an orange peel appearance.
• Inflammation and tightening of the skin.
• Burning, itching or severe sharp pains in areas of involvement, typically the extremities or the trunk. The face is almost never involved.
• Skin thickening that inhibits movement, resulting in permanent shortening of muscles, tendons or scar tissue. Patients may experience muscle weakness. Others may be unable to walk, or fully extend the joints of their arms, hands, legs, and feet.
• Symmetrical skin lesions, with zones between the ankles and thighs most common, followed by the wrist and upper arms.
• Hand and foot swelling with blisterlike lesions; yellow plaques in or near the eyes.
• Pain in the hips and ribs.

Some patients with NFD have calcium abnormalities such as traumatic calcinosis cutis, osseous metaplasia, calciphylaxis and metastatic calcification — which involve depositing of calcium into body tissues. These disorders are different than NFD, but can appear to be very similar, and, in some patients, can co-exist with NFD. Some patients with NFD experience blood clots and deep vein thrombosis and the disease is also associated with kidney transplant failure and rapid onset of kidney disease with swollen extremities.