Scleroderma (skleer-oh-DUR-muh) is a group of rare, progressive diseases that involve the hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body.
Localized scleroderma affects only the skin. Systemic scleroderma also harms internal organs, such as the heart, lungs, kidneys and digestive tract.
Scleroderma can happen to anyone at any time in any geographical area. However, the disease affects women more often than men and most commonly occurs between the ages of 30 and 50. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn't considered contagious, but it can greatly affect self-esteem and the ability to accomplish everyday tasks.
Scleroderma is a progressive disorder characterized by thickening and tightening of the skin — especially on the arms, face and hands — which results in loss of flexibility.
Scleroderma symptoms vary, depending on which organ systems are involved. Diagnosis can be difficult because some of the early symptoms are common in the general population and aren't always associated with scleroderma. The most prevalent signs and symptoms of scleroderma include:
With localized scleroderma, the first signs of disease may be the presence of Raynaud's phenomenon and may develop several years before you notice any other symptoms.
With systemic scleroderma, skin changes may occur suddenly and progressively worsen during the first one to two years of the disease. After that, changes level off or subside, and sometimes even resolve on their own without treatment.
Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.
Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.
Several factors appear to increase the risk of certain types of scleroderma:
Race and ethnicity
Scleroderma complications range from mild to severe. Some may even become life-threatening.
Preparing for your appointment
You'll probably first bring your symptoms to the attention of your family doctor, who may refer you to a rheumatologist — a doctor specializing in the treatment of arthritis and other diseases of the joints, muscles and bone. Because scleroderma can affect many organ systems, you may need to see a variety of medical specialists.
What you can do
What to expect from your doctor
Tests and diagnosis
Your doctor may conduct the following tests:
Based on the outcome of your initial evaluation, your doctor may also recommend other diagnostic tests to identify any lung, heart, kidney or gastrointestinal complications accompanying scleroderma.
Treatments and drugs
Scleroderma has no known cure — no drug will stop the overproduction of collagen. But the localized variety of scleroderma sometimes resolves on its own. And a variety of medications can help control the symptoms of scleroderma or help prevent complications.
Lifestyle and home remedies
You can take a number of steps to help manage your symptoms of scleroderma:
Meditation and relaxation techniques can help you cope with the frustrations of scleroderma, and help relieve pain and fatigue.
Coping and support
As is true with other chronic diseases, living with scleroderma can place you on a roller coaster of emotions. Here are some suggestions to help you even out the ups and downs:
If scleroderma makes it difficult for you to do things you enjoy, ask your doctor about ways to get around the obstacles.
Keep in mind that your physical health can have a direct impact on your mental health. Denial, anger and frustration are common with chronic illnesses.
At times, you may need additional tools to deal with your emotions. Professionals, such as therapists or behavior psychologists, may be able to help you put things in perspective. They can also help you develop coping skills, including relaxation techniques.
Joining a support group, where you can share experiences and feelings with other people, is often a good approach. Ask your doctor what support groups are available in your community.
Last Updated: 2010-10-16
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