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Scleroderma is a chronic disease characterized by hardened or thickened skin. This thickening is caused by a build-up of collagen under the skin which may also occur near other organs. The collagen can form scar tissue (fibrosis) and affect the function of the organs. Scleroderma is considered an autoimmune disease, where the immune system recognizes the body’s own tissues as an invader and attacks it.
Scleroderma has two major forms. The first, called localized scleroderma, only affects the skin and the tissue below it. The second, known as systemic scleroderma, affects the skin more widely and also can affect other organs. The systemic form of the disease involves a progressive hardening of tissues and organs and often leads to digestive, respiratory and circulatory system complications.
In its visible manifestations, systemic scleroderma causes thickened, tightened and shiny patches of skin. It can swell the fingers so they are difficult to use and swell the mouth enough to make eating troublesome. When systemic scleroderma causes collagen build-up in vital organs, other symptoms such as joint pain, shortness of breath and difficulty swallowing may occur.
The skin symptoms of systemic scleroderma may be unpleasant and sometimes disfiguring. The systemic complications that some patients experience may affect their lifestyles and sometimes result in death. Treatment (e.g., anti-inflammatory and immunosupressant medications) is available to manage the symptoms, but there is no cure for systemic scleroderma.
An estimated 300,000 people in the United States are believed to have scleroderma, including as many as 100,000 who have the systemic form of this disease, according the Scleroderma Foundation. Approximately three to four times more women than men are afflicted with scleroderma. It usually affects individuals between the ages of 30 and 50. |