Scleroderma (pronounced SKLEER-oh-der-ma) is Greek for "hard skin." 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, it can injure the body's digestive, respiratory, urinary, and circulatory systems.
Avascular necrosis (AVN), also called osteonecrosis, aseptic necrosis, or ischemic bone necrosis, is a condition that occurs when there is loss of blood to the bone. Because bone is living tissue that requires blood, an interruption to the blood supply causes bone to die. If not stopped, this process eventually causes the bone to collapse.
Avascular necrosis most commonly occurs in the upper leg. Other common sites are the upper arm, knees, shoulder, and ankles.
There are many types of scleroderma, and it can look very different in different people. Doctors 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. There are more cases in the United States than in Europe or Japan. No one race or ethnic group is affected more than another, although women have scleroderma in much greater numbers than men.
Women have 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 get scleroderma.
What Causes Scleroderma?
The cause of scleroderma is not known. For some unknown reason, the immune system turns against the body and causes too much collagen to be made. Collagen is one of the building blocks of skin. When too much collagen is made by the body, it causes other tissues to become thick and lose flexibility and affects their ability to work properly.