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Scleroderma is a type of progressive autoimmune rheumatic disease that affects about 300,000 Americans. When a person has scleroderma, their body’s immune system destroys normal, healthy tissues, replacing them with scar tissue. The name comes from the Greek words “sclero” meaning hard and derma meaning skin. It is marked by a thickening and tightening of the skin and connective tissues, but may also involve the lungs, esophagus, kidneys and digestive tract. Women are affected more than men and have an onset between the ages of 30 and 50.
There are two types: localized and systemic. Localized includes hard, oval-shaped patches on the skin and lines or streaks of thickened skin on the arms, legs, or face. The underlying problem is the overproduction of collagen or scar tissue that is confined to certain areas of the skin. Systemic involves multiple body parts or systems. In systemic scleroderma, three processes are in play: overproduction of collagen (which affects not only the skin but also the heart, lungs and lining of the gastrointestinal tract), blood vessel abnormalities in which the vessels become damaged and narrowed, and immune system dysfunction.
Scleroderma is not contagious and does not cause cancer. The major causes of death in people with the disease are pulmonary hypertension, pulmonary fibrosis and kidney failure.
Scientists aren’t sure what causes scleroderma. All that is known currently is that it involves an overproduction and accumulation of collagen, a fibrous type of protein that makes up your body’s connective tissues including the skin. It is an autoimmune disease, meaning that the immune system attacks healthy tissues, including the skin and other organs, but it is unclear why this happens.
People with systemic scleroderma tend to have family members with other autoimmune diseases (such as lupus), suggesting a genetic component; however, most people with scleroderma have no family history of the disease or other autoimmune diseases. It is thought that environmental factors may also trigger the disease – for example, exposure to silica in coal mining or sand blasting has been linked to systemic scleroderma.