Scleroderma is a relatively rare disease that affects the connective tissues of the body, causing hardening and scarring of skin and sometimes other organs. The exact number of people who have it is not known. Estimates of the number of people with scleroderma in the United States range from 28 to 265 per 100,000 population. Between 10 and 20 new cases of scleroderma per million people may be diagnosed each year.

Scleroderma can begin at any age, but onset usually occurs in patients between the ages of 30 and 50. Children account for less than three percent of diagnosed cases of scleroderma. Scleroderma in children is usually a limited or localized form of the disease.

Scleroderma occurs in women more commonly than in men. About three to eight women are diagnosed with scleroderma for every man who receives the diagnosis. Although the disease affects more women, some research suggests that the death rate associated with scleroderma is greater for men.

The number of cases of scleroderma varies by geographic region. The disease is more common in the United States than in Europe or Japan. Ethnic background also has a bearing on the incidence of scleroderma. The disease occurs more commonly among African-American women than among Caucasian women of the same age.

Research to date has been unable to identify a single gene or environmental agent responsible for scleroderma. It is likely that the disease is triggered by a complex interaction of many factors.

Scientists have identified a gene that may increase a person's risk for scleroderma. Clusters of the disease have been noted among small groups in Tennessee, North Carolina, and Oklahoma, so researchers are studying the family lines within these clusters to better understand the possible genetic influence on scleroderma.

Recent concerns about silicone breast implants led researchers to study this risk factor more closely. So far, research suggests that silicone breast implants do not appear to increase the risk for scleroderma. Likewise, implants do not appear to increase the risk of the disease in breast-fed babies.

Researchers also studied exposure to other sources of silicone, including glues, sealants, and simethicone-containing antacids. Such exposure does not appear to increase the risk of scleroderma.

Other factors that may increase the risk for scleroderma include high cumulative exposure to organic solvents and vinyl chloride in manufacturing plants. Contaminated rapeseed oil, the supplement L-tryptophan, and some drugs such as bleomycin have also been implicated as possible triggers of scleroderma or scleroderma-like syndromes in some people.

References

First published April 1, 2000
Last updated September 10, 2003
Copyright © 2000 Accordant Health Services, Inc. All Rights Reserved.

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