Scleroderma is a widespread connective tissue disease that 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. Women get scleroderma more often than men do. Risk factors include work exposure to silica dust and polyvinyl chloride.
Diffuse scleroderma can overlap with other autoimmune diseases, including systemic lupus erythematosus and polymyositis. In such cases, the disorder is referred to as mixed connective disease.
Localized scleroderma usually affects only the skin on the hands and face. Its course is very slow, and it rarely, if ever, spreads throughout the body (becomes systemic) or causes serious complications.
Systemic scleroderma is also called systemic sclerosis. This form of the disease may affect the organs of the body, large areas of the skin, or both. This form of scleroderma has two main types: limited and diffuse scleroderma.
Skin symptoms may include:
Bone and muscle symptoms may include:
Breathing problems may include:
Digestive tract problems may include:
Examination of the skin may show tightness, thickening, and hardening.
Blood tests may include:
Other tests may include:
There is still no treatment for the disease process of scleroderma. However, certain drugs and treatments help combat the complications.
Drugs used to treat scleroderma include:
Other treatments for specific symptoms may include:
Treatment usually includes a combination of physical therapy and skin and joint protection techniques (for example, avoiding cold in the case of Raynaud's phenomenon).
See: Scleroderma - resources
In most patients, the disease slowly gets worse. People who only have skin symptoms have a better outlook. Death may occur from gastrointestinal, heart, kidney, or lung complications.
One type of scleroderma, called limited scleroderma, involves only problems of the skin of the hands and face. It gets worse very slowly and usually does not affect other parts of the body.
Systemic scleroderma can involve many organs in the body. In some people, it will progress slowly and not involve any organs in the body. In other people, organs such as the lungs, kidneys, intestines, gallbladder, and heart become involved.
For some people, symptoms and problems develop quickly over the first few years, and continue to get worse. Others get worse much more slowly. Lung problems are the most common cause of death in patients with scleroderma.
Call for an appointment with your health care provider if:
There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride.
CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Morphea, linear