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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.

The problems caused by limited scleroderma may be minor. Sometimes, 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.

Symptoms Causes Risk factors Complications

While some varieties of scleroderma occur rapidly, signs and symptoms of limited scleroderma usually develop gradually. They include:

  • Tight, hardened skin. In limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and neck. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.
  • Raynaud's phenomenon. This condition occurs when small blood vessels in your fingers and toes go into spasms in response to cold or emotional stress, blocking the flow of blood. In most people, the skin turns white before becoming blue, cold and numb. When circulation improves, the skin usually reddens and may throb or tingle. Raynaud's phenomenon is often one of the earliest signs of limited scleroderma, but many people have only Raynaud's and never develop scleroderma.
  • Red spots or lines on skin. These small red spots or lines (telangiectasias) are caused by the swelling of tiny blood vessels near the skin's surface. They are not painful and occur primarily on the hands and face.
  • Bumps under the skin. Limited scleroderma may cause tiny calcium deposits (calcinosis) to develop under your skin, mainly on your elbows, knees and fingers. You can see and feel these deposits, which sometimes may be tender or become infected.
  • Swallowing difficulties. People with limited scleroderma commonly experience problems with their esophagus — the tube that connects the mouth and stomach. Poor functioning of the muscles in the upper and lower esophagus can make swallowing difficult and allow stomach acids to back up into the esophagus, leading to heartburn, inflammation and scarring of esophageal tissues.

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.

  • Sex. Women are far more likely to develop limited scleroderma than men are.
  • Race. In the United States, limited scleroderma affects blacks more often than whites. Choctaw Native Americans also have higher rates of limited scleroderma than other races.
  • Genetic factors. If someone in your family has an autoimmune disease — such as lupus, rheumatoid arthritis or Hashimoto's disease — you have an increased risk of developing limited scleroderma.
  • Exposure to toxins. Certain toxic substances — such as polyvinyl chloride, benzene, silica and trichloroethylene — may trigger scleroderma in people with a genetic predisposition to the disease.

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.

  • Gastrointestinal problems. Changes in the functioning of esophageal muscles can cause difficulty swallowing and chronic heartburn. When limited scleroderma affects your intestine, you may experience constipation, diarrhea, bloating after meals, unintended weight loss and malnutrition.
  • Ulcers on fingers and toes. Severe Raynaud's phenomenon can obstruct blood flow to your extremities and may cause ulcers of the fingers and toes. These ulcers can be difficult to heal. Additionally, abnormal or narrowed blood vessels combined with severe Raynaud's phenomenon can lead to gangrene of fingers or toes, which may require amputation.
  • Lung damage. Limited scleroderma can cause a variety of problems with your lungs. In some cases, excess collagen collects in the tissue between the lungs' air sacs, making the lung tissue stiffer and less able to work properly. Increased blood pressure in the arteries between your heart and lungs makes the heart work harder and eventually weakens it.
  • Heart problems. Scarring of heart tissue can lead to abnormal heart rhythms (arrhythmias) and, in rare cases, to an inflamed heart muscle (myocarditis).
  • Dental problems. Severe tightening of facial skin can make it difficult to open your mouth wide enough to brush your teeth. Acid reflux can destroy tooth enamel, and changes in gum tissue may cause your teeth to become loose or even fall out.
  • Dry eyes and mouth. Many people with limited scleroderma experience very dry eyes and mouth.
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