Scleroderma is a disease that causes thickening and tightness of the skin. Often the swallowing muscles become stiff. Blood vessels and other organs may also become stiff. For most people the effects of scleroderma are mild and limited to the skin and swallowing muscles. This form of the disease is called localized scleroderma.
Systemic sclerosis is the term used to describe the severe form of scleroderma, which affects many organs in the body. In this form of the illness, the same hardness that affects the skin and swallowing muscles can severely damage or destroy the kidneys, heart, lungs, and organs of the digestive tract.
Scleroderma usually develops in middle age.
Each organ affected by scleroderma, including the skin, goes through a sequence of destructive changes: from inflammation, to hardening, to complete scarring. In severe cases some organs finally stop working.
In most cases this destruction appears to be an autoimmune problem. This means the body mistakenly attacks its own tissue. Over 90% of people with systemic sclerosis appear to have antibodies in their blood that attack their cells and tissues. Doctors do not know why this happens.
In the milder, localized form of scleroderma the destructive changes sometimes appear to be brought on by occupational exposure. An example of such exposure is long-term use of a jackhammer with constant vibration.
Changes in the skin are among the most common symptoms of scleroderma. The fingers and other patches of skin become hard and numb. These areas of skin may appear smooth and slightly swollen. The skin may become tight, making it hard to move the joints, especially the wrists and elbows. This tightness may occur around the mouth as well.
Another very common symptom is a reaction called Raynaud's phenomenon. Raynaud's phenomenon can be brought on by cold, emotion, or stress. When this happens, the fingers and sometimes toes, ears, and nose are very sensitive to cold temperatures. They react to cold by turning pale, then blue, and eventually red. They feel tingly or painful.
Other possible symptoms include:
In localized scleroderma the symptoms tend to develop slowly over time. In systemic sclerosis the symptoms may develop slowly, or they may occur suddenly and get worse quickly.
Your health care provider will ask about your symptoms and do a physical exam. He or she will examine your skin.
Possible tests for scleroderma include:
Other tests may be done to check organs such as your liver, kidneys, heart, or lungs.
There is no single drug treatment for scleroderma. Sometimes medicines that suppress the immune system are used. Steroids, which are often helpful in other inflammatory or autoimmune diseases, do not seem to be helpful in scleroderma. Most treatment for scleroderma is directed at specific symptoms.
Raynaud's phenomenon may be helped with medicines called calcium channel blockers, which help the circulation. Another medicine for Raynaud's is nitroglycerin ointment, which is rubbed onto the skin. Even with medicine, however, it is very important to dress properly for the weather and avoid letting your skin get cold. Also, do your best to avoid stressful situations.
Treatment of scleroderma, and especially systemic sclerosis, includes frequent checks of your blood pressure. Prompt treatment of any increases in blood pressure with medicine may help prevent or slow damage to the kidneys. It may also help prevent other complications of high blood pressure, such as heart disease and stroke.
Many drugs are being studied to see if they will prevent or control the effects of scleroderma. Other treatments under study include light therapy (ultraviolet A) and bone marrow transplants (BMT). The goal of BMT is to replace old, problem-causing cells with new, healthy cells. Unfortunately, BMT has some serious side effects and complications.
Scleroderma is a permanent condition, although the symptoms may come and go. Unfortunately, in most cases the symptoms get worse with time. However, much research is being focused on autoimmune diseases. Treatments may be found soon that will help slow or stop the course of systemic sclerosis.
There is no known way to prevent scleroderma.
Disclaimer: This content is reviewed periodically and is subject to change as new health information becomes available. The information provided is intended to be informative and educational and is not a replacement for professional medical evaluation, advice, diagnosis or treatment by a healthcare professional.
HIA File BJM3231F.HTM Release 9.0/2006
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