Scleroderma

What is scleroderma?

Scleroderma is a disease that causes parts of the body to get thick and hard. It may affect the skin, joints, muscles, blood vessels, and organs such as the kidneys, heart, and lungs.

For most people the effects of scleroderma are mild and limited to the skin and the muscles used for swallowing. This form of the disease is called localized scleroderma. A more severe form of scleroderma is called systemic sclerosis. It 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. In severe cases some organs eventually stop working.

Scleroderma usually develops in middle age. Scleroderma is a permanent condition, although the symptoms may come and go. Unfortunately, in most cases the symptoms get worse with time.

What is the cause?

The exact cause of scleroderma is not known. It appears to be an autoimmune disease. This means that your body’s defense against infection (your immune system) attacks your own tissue–for example, your skin, blood vessels, or joints.

What are the symptoms?

Changes in the skin are among the most common symptoms of scleroderma. Your fingers and other patches of skin get hard and numb. These areas of skin may look smooth and slightly swollen. Your skin may get tight, making it hard to move your joints, especially your fingers, wrists, and elbows. You may have tightness around your mouth as well.

Another very common symptom is a reaction called Raynaud’s phenomenon. Raynaud’s can be brought on by cold temperatures, 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. The skin feels cold and then may throb or feel numb, tingly, or painful as it warms up or as stress is relieved. Each attack of symptoms usually lasts for just a few minutes, but some may last more than an hour.

Other symptoms may include:

  • Trouble swallowing pills or some foods, such as meat
  • Heartburn
  • Weakness
  • Shortness of breath or trouble taking deep breaths

The symptoms of localized scleroderma tend to develop slowly over time. The symptoms of systemic sclerosis may develop slowly or they may happen suddenly and get worse quickly.

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical history and examine you.

Possible tests for scleroderma include:

  • Skin biopsy, which is the removal of a small sample of tissue for testing.
  • Blood tests
  • 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?

Treatment doesn’t cure the disease but helps to ease the symptoms. Treatment may include medicines to:

  • Help control your immune system
  • Treat Raynaud’s symptoms
  • Lower blood pressure

Prompt treatment of any increases in your blood pressure may help prevent or slow damage to your kidneys. It can help prevent kidney disease, heart disease, and stroke.

Research for scleroderma treatment is ongoing. Many drugs are being studied to see if they will prevent or control the effects of scleroderma.

How can I take care of myself?

  • Follow your healthcare provider’s instructions for taking prescribed medicines.
  • Stay as active as you can with regular exercise or physical therapy, as recommended by your healthcare provider.
  • Avoid overwork and getting overly tired. Get plenty of rest.
  • Avoid cold temperatures, including very cold air conditioning. Be sure to dress properly for the weather and avoid letting your skin get cold. Also, do your best to avoid stressful situations.
  • If you smoke, try to quit. Talk to your healthcare provider about ways to quit smoking. 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 dry and cracking skin.
  • Learn skills to cope with stress and tiredness, such as meditation and relaxation techniques.
  • Ask for a referral to an occupational therapist (OT) if you are having trouble using your hands or drinking and swallowing. OTs can help with special techniques and tools.
  • Ask your healthcare provider:
    • How and when you will hear your test results
    • How long it will take to recover
    • What activities you should avoid and when you can return to your normal activities
    • How to take care of yourself at home
    • What symptoms or problems you should watch for and what to do if you have them
  • Make sure you know when you should come back for a checkup.
Developed by RelayHealth.
Adult Advisor 2015.1 published by RelayHealth.
Last modified: 2013-10-24
Last reviewed: 2013-07-31
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
Copyright ©1986-2015 McKesson Corporation and/or one of its subsidiaries. All rights reserved.

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