Scleroderma

Scleroderma is a slowly progressive disease characterized by deposits of fibrous connective tissue in the skin and often the internal organs.

What Is Scleroderma?

Scleroderma is a slowly progressive disease characterized by deposits of fibrous connective tissue in the skin and often the internal organs, by hand and foot pain during exposure to cold, and by tightening and thickening of the skin. Localized scleroderma primarily affects the skin, whereas more widespread scleroderma also affects such internal organs as the lungs, liver, and heart, kidneys, and those organs in the gastrointestinal tract.

What Causes Scleroderma?

As of 2009, scientist were not sure what causes scleroderma. It was thought to be a typc of autoimmune disease. Ordinarily, the function of the immune system is only directed against foreign agents such as viruses, bacteria, or fungi, which threaten the body's health. However, in the case of an autoimmune disease, the immune system accidentally becomes confused about what is "foreign" and what is "self." The immune system begins to marshal its resources to attack the organs and tissues of the body, causing damage and destruction. In the case of scleroderma, it appears that the immune system's attack on connective tissue prompts an inflammatory response and the overproduction of collagen, which is an insoluble fibrous protein and the chief component of connective tissue. The accumulation of excess collagen causes the signs and symptoms of scleroderma.

Who Gets Scleroderma?

Scleroderma tends to strike people between 30 and 50 ycars of age. Women are four times as likely as men to develop this condition. Only about 4 to 12 individuals per 12 million people are diagnosed yearly, although more mild cases may occur without diagnosis. People of African descent are more likely to have the disease than people of European descent, and African-Americans are also more likely to have severe lung disease as a component of their condition. Other risk factors for the development of scleroderma include exposure to silica dust, paint thinners, and chemotherapy agents.