Scleroderma is an autoimmune disorder that affects connective tissue and involves changes in the skin, blood vessels, muscles and internal organs.
The cause of scleroderma is unknown. People with this condition have a buildup of a substance called collagen in the skin and other organs. This buildup leads to the symptoms of the disease.
The disease usually affects people 30 to 50 years old and is diagnosed more frequently in women than men. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.
Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus (SLE) and polymyositis. In such cases, the disorder is referred to as “mixed connective disease.”
Some types of scleroderma affect only the skin, while others affect the whole body. Localized scleroderma usually affects only the skin on the hands and face. It develops slowly, and rarely, if ever, spreads throughout the body or causes serious complications. Systemic scleroderma, or sclerosis, may affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types of systemic scleroderma: Limited disease (CREST syndrome) and diffuse disease.
There is no specific treatment for scleroderma. Some people with scleroderma have symptoms that develop quickly over the first few years and continue to get worse. In most patients, however, the disease begins and worsens slowly. People who only have skin symptoms have a better outlook. Widespread (systemic) scleroderma can damage the heart, kidney, lungs, or GI tract, which may cause death.
Lung problems are the most common cause of death in patients with scleroderma.