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Scleroderma
Alternate Names : Progressive Systemic Sclerosis
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Scleroderma is a widespread autoimmune disorder. It causes the skin and other body parts to slowly degenerate, thicken, and stiffen. An autoimmune disorder is one in which the body produces antibodies against its own tissues for no known reason.
What is going on in the body?
Scleroderma results from an overproduction of collagen, which is the main supportive protein in the body. The excess collagen affects tiny blood vessels and almost every organ.
A person may develop either a localized or a systemic form of scleroderma. The localized form is most common in children. It usually involves a few places on the skin, soft tissues, or muscles. The localized form rarely, if ever, progresses to the systemic form of scleroderma.
The systemic form affects skin, muscles, and soft tissues. It also affects other body organs and structures, such as the following:
blood vessels
gastrointestinal, or GI, tract
heart
kidneys
lungs
What are the causes and risks of the condition?
The exact cause of scleroderma is unknown. It is generally thought to be an autoimmune disorder.
New research findings suggest that autoimmune disorders may be triggered by a transfer of cells between the fetus and the mother during pregnancy. Women with scleroderma have more fetal cells in their blood decades after a pregnancy than women who don't have scleroderma. While further research is needed to substantiate these findings, the study does offer an explanation for the much higher incidence of autoimmune disorders in women than in men.
People heavily exposed to certain chemicals or substances may be at higher than normal risk for scleroderma. These substances include the following:
detergent
herbicides
plastics
silica
silicone prostheses
various drugs, such as cocaine and amphetamines
Mild cases may develop from occupational repetitive stresses on the hand, such as from working with a jackhammer.
This condition usually occurs between the ages of 30 and 50, but it may also occur in children. It is four times more common in women than men but can affect either sex.
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Author: Gail Hendrickson, RN, BS Reviewer: Barbara Mallari, RN, BSN, PHN Date Reviewed: 08/06/01
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