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Nephrogenic Systemic Fibrosis

Symptoms

Some signs and symptoms of nephrogenic systemic fibrosis include:

  • Hardened masses on the skin, thickening and hardening of the skin, and darkening (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 and tendons. Some people 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 blister-like lesions; yellow plaques in or near the eyes.
  • Pain in the hips and ribs.

Some people with nephrogenic systemic fibrosis have disorders in which calcium is deposited into body tissue — such as traumatic calcinosis cutis, osseous metaplasia, calciphylaxis and metastatic calcification. These disorders are different from nephrogenic systemic fibrosis, but can appear very similar and, in some people, can coexist with nephrogenic systemic fibrosis.

Some people experience blood clots and deep vein thrombosis. Nephrogenic systemic fibrosis is also associated with failure of kidney transplant and rapid onset of kidney disease with swollen extremities.

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