Scleroderma is a rare but debilitating autoimmune disease where the skin thickens and tightens, where the body's joints allow for less and less bending. Hands, elbows, and knees all become stiff. Hardening may also affect internal organs - heart, lungs, and kidneys, plus gastrointestinal tract and vascular system.
Another rare skin hardening condition is NSF or nephrogenic systemic fibrosis caused when people with kidney disease or on kidney dialysis receive a gadolinium contrast imaging injection with their MRI. People who receive MRIs but who do not suffer from kidney disease don't get NSF. NSF may also affect internal organs. Pneumonia is the number one cause of death for people with NSF.
It is unknown what causes scleroderma. Researchers believe scleroderma may be caused by a genetic predisposition interacting with environmental stimulus.
According to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, the immune system is believed to stimulate cells called fibroblasts, causing them to produce too much collagen. As the collagen builds, thick connective tissue within the skin and internal organs can interfere with their function.
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Localized scleroderma can take the form of reddish patches of skin that thicken into oval-shaped areas. As the disease progresses, the skin grows thicker even to the point of changing a person's appearance. The thickening can also spread, becoming systemic. The fingers, hands, face, lower arms, and legs are the only places affected if the spread is limited, but people also can suffer a range of symptoms including:
- The formation of calcium deposits in the connective tissues, usually found on the fingers, hands, face, and trunk, and on the skin above the elbows and knees.
- The small blood vessels of the hands or feet contract in response to cold or anxiety; a patient may suffer gangrene.
- Swallowing difficulty or chronic heartburn happens when the esophagus loses normal movement.
- Difficulty bending fingers caused by excessive collagen deposits
- Small red spots appear on the hands and face caused by swelling of tiny blood vessels.
In the most serious cases, internal organs are affected. Kidneys quit working. Lungs stiffen, making it hard to take a breath. Hearts are unable to pump as efficiently. Doctors treat the affected organs. Currently, there is effective kidney treatment for scleroderma.
There is no effective treatment for NSF.