Limited scleroderma (CREST syndrome)
Limited scleroderma (CREST syndrome)
Limited scleroderma, or CREST syndrome, is one subtype of scleroderma — a condition that literally means "hardened skin."
The skin changes associated with limited scleroderma typically occur only in the lower arms and legs, and sometimes the face and throat. Limited scleroderma can also affect your digestive tract and can cause serious heart and lung disorders.
The problems caused by limited scleroderma may be minor. In some cases, however, the disease affects the lungs or heart, with potentially serious results. Limited scleroderma has no known cure, and treatments focus on managing symptoms and preventing serious complications.
While some varieties of scleroderma occur rapidly, signs and symptoms of limited scleroderma usually develop gradually. They include:
In limited scleroderma, dry ulcers can develop on fingers and toes. The skin can become taut and shiny. Also, dilated blood vessels can collect on the surface of the skin. ...
Raynaud's disease is a vascular disorder that causes intermittent interruption of blood flow to the extremities. The affected body part may turn white or blue and feel cold and numb until circulation ...
Telangiectasias are permanently dilated small blood vessels. They can develop externally in the skin and may appear as a group of red lines. ...
Limited scleroderma is believed to be an autoimmune disorder — one in which your immune system turns against your own body. In limited scleroderma, the immune system appears to stimulate the production of too much collagen, a key component of connective tissue. This overproduction of collagen builds up in the skin and internal organs, impairing their function.
The visible signs of limited scleroderma — tight, thick skin on your fingers, hands and face — can affect the way you feel about your appearance; make everyday tasks, such as opening a jar or shaving, more difficult; and even affect your speech. But the most serious complications tend to occur beneath your skin.
Ulcers on fingers and toes
Dry eyes and mouth
Preparing for your appointment
Because limited scleroderma can affect so many different parts of your body, you may need to be seen by doctors in a variety of specialties, including:
What you can do
Since your time with your doctor is limited, preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For limited scleroderma, some basic questions to ask your doctor include:
What to expect from your doctor
Tests and diagnosis
Like other unusual and complex disorders, limited scleroderma can be difficult to diagnose. Signs and symptoms vary widely and often resemble those of other connective tissue and autoimmune diseases. Further complicating matters is that limited scleroderma sometimes occurs with other autoimmune conditions — such as polymyositis, lupus and rheumatoid arthritis.
Tests that may be used in the diagnosis of limited scleroderma include:
In addition to tests that may help diagnose limited scleroderma itself, your doctor may recommend additional tests to identify any lung, heart or gastrointestinal complications.
Treatments and drugs
Limited scleroderma has no known cure. Treatment focuses on relieving signs and symptoms and preventing complications.
Lifestyle and home remedies
Change eating habits
Protect your skin
Practice good oral hygiene
To help boost blood flow to extremities, you might try hypnosis or biofeedback, a technique that teaches you to control certain body responses.
Coping and support
Because limited scleroderma can affect your appearance and your ability to perform simple tasks, your self-esteem may suffer. It's common for people with limited scleroderma to become depressed.
Talking with a counselor may help you come to grips with the changes caused by this disease. Communicating with people facing the same illness, either through online or in-person support groups, may also be helpful.
Last Updated: 2011-06-02
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