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Understanding Scleroderma - The Basics
Scleroderma (pronounced SKLEER-oh-der-ma) is a term that comes from the Greek for "hard skin." It's classified as both a connective tissue disorder and a rheumatic condition. In scleroderma, the skin gradually tightens and thickens or hardens. It loses its ability to stretch.
Tiny blood vessels throughout the body also may be affected, causing widespread damage to internal organs. Although the disease usually affects the hands, face, and feet, impaired blood flow can eventually injure the body's digestive, respiratory, and circulatory systems.
Scleroderma can look very different in different people. There are many subtypes of this condition. Doctors generally classify scleroderma as either localized or systemic, depending on the degree to which the skin is affected. The widespread form of the disease is often called systemic sclerosis and can be life-threatening.
An estimated 300,000 Americans have scleroderma. The incidence appears to be higher in the United States than in Europe or Japan and varies across geographic regions. No one race or ethnic group is affected more than another, although women have scleroderma in much greater numbers.
Women develop localized forms of the disease three times as frequently as men, and more than 80% of people with systemic scleroderma are women aged 30 to 50. Rarely, children can also develop scleroderma. In the past two decades, longevity has improved for people with systemic scleroderma as better treatments have been developed.
What Causes Scleroderma?
The main feature of scleroderma is excessive production of collagen. Collagen is one of the building blocks from which skin is made. Usually, it makes the skin supple. But when too much collagen accumulates, it causes various tissues to become thick and lose flexibility.
The underlying cause of scleroderma remains unclear. For some unknown reason, the immune system turns against the body and results in the overproduction of collagen. Current research focuses on the interplay between the immune system, the vascular (blood vessel) system, and the growth of connective tissue. Studies with twins suggest that this disease is not inherited.
WebMD Medical Reference
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