Scleroderma

What is scleroderma?

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 more 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.

How does it occur?

Each organ affected by scleroderma, including the skin, goes through a sequence of destructive changes: from inflammation, to hardening, and then to complete scarring. In severe cases some organs eventually stop working at all.

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.

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. 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 of scleroderma include:

• trouble swallowing pills or solid foods, such as meat
• heartburn
• weakness
• shortness of breath or trouble taking deep breaths because the skin of the chest has tightened or the lungs are becoming scarred.

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.

How is it diagnosed?

Your healthcare 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 to see how well you can swallow.

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

How is it treated?

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.

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 will help slow or stop the course of systemic sclerosis.

How can I help take care of myself?

• Follow your healthcare provider's instructions for taking prescribed medicines.
• Stay as active as possible with regular exercise or physical therapy, as recommended by your healthcare provider.
• Avoid overwork and getting overly tired. Get plenty of rest.
• Avoid cold and air conditioning.
• Do not smoke. Avoid being around smoke.
• Ask your provider about heat therapy for stiff joints.
• Avoid fingersticks for blood tests.
• Use lotions, ointments, and bath oils to prevent skin dryness and cracking.
• Tell your healthcare provider about any new symptoms right away.
• Learn skills to cope with stress and tiredness, such as meditation and relaxation techniques.

How can I prevent scleroderma?

There is no known way to prevent scleroderma.