What is scleroderma?

Scleroderma is a disease that causes hardening and tightness of the skin. Often the swallowing muscles, blood vessels, and other organs are also hardened. For most people the effects of scleroderma are mild and limited to the skin and swallowing muscles.

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 organs of the digestive tract, kidneys, heart, and lungs.

Scleroderma usually develops in middle age.

How does it occur?

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. Doctors do not yet understand what exactly causes this destruction. In the milder form of scleroderma these 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.

However, for most people with limited scleroderma, and for virtually all people with systemic sclerosis, the illness appears to be an autoimmune disease. 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 own cells and tissues.

What are the symptoms?

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. 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. Raynaud's phenomenon can be brought on by emotion or stress, as well as by cold.

Other possible symptoms include:

• trouble swallowing pills or solid foods, such as meat

• heartburn

• weakness

• shortness of breath or difficulty taking a deep breath because the skin of the chest has tightened or the lungs are becoming scarred.

In limited scleroderma the symptoms tend to develop slowly over time. In systemic sclerosis the symptoms may develop slowly or they may occur suddenly.

How is it diagnosed?

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:

• skin biopsy

• blood tests, such as tests for ANA (antinuclear antibody) and ACA (anticentromere antibody), which look for signs that the body is rejecting its own tissues

• special x-rays during swallowing.

Other tests may be done to check other organs, such as the liver, kidneys, heart, or lungs.

How is it treated?

There is no single drug treatment for scleroderma. The treatment depends on the symptoms and the parts of the body that are affected.

The most important thing you can do for Raynaud's phenomenon is to dress properly for the weather and avoid letting your skin get cold. Also, do your best to avoid stressful situations.

At least three kinds of medications are sometimes used to treat severe Raynaud's symptoms. They all improve circulation. One such medicine, nitroglycerin ointment, can be rubbed onto the skin. The other medications are taken by mouth.

Treatment of scleroderma, and especially systemic sclerosis, includes frequent checks of your blood pressure. Prompt treatment of any increases in blood pressure may help prevent or slow damage to the kidneys.

Steroids (for example, Prednisone) are sometimes used to reduce inflammation of the skin, joints, or muscles. However, steroids cannot prevent damage to the organs.

Drugs that have been used in the past to try to control the symptoms of scleroderma and to stop organ damage include D- penicillamine and colchicine. Both of these drugs sometimes help reverse skin changes caused by scleroderma. However, they do not appear to have any effect on other organs.

How long will the effects last?

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 help slow or stop the course of systemic sclerosis.

How can I prevent scleroderma?

There is no known way to prevent scleroderma or systemic sclerosis.