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Scleroderma

CREST syndrome

A less severe form of scleroderma is called CREST.

The CREST syndrome represents symptoms including calcium skin deposits; Raynaud's phenomenon (a condition in which the blood vessels of the fingers and toes go into spasm when triggered by factors such as cold, stress, or illness; the result is cold, painful, or numb fingers and toes which in severe cases may become gangrenous); esophageal dysfunction (problems with the esophagus, the tube between the mouth and the stomach); sclerodactyly (skin damage on fingers); and telangiectasia (spider veins), limits skin damage to the fingers.

However, this disease, when coupled with pulmonary hypertension (elevated blood pressures within the lungs), can lead to heart and respiratory failure.

What is scleroderma?

Scleroderma can be either a localized disease or a disease that affects the whole body. When it affects your whole body it is also called systemic sclerosis or systemic scleroderma. Scleroderma is a chronic, degenerative disease causing abnormal growth of the connective tissue that affects the joints, skin, and internal organs. Scleroderma is also associated with blood vessel abnormalities.

Scleroderma is thought to be an autoimmune disease, meaning that the body's immune system turns on itself. Although genes play a role in the disease, it is not passed on from parents to children. In addition, unknown environmental factors likely play a role.

What are the symptoms of scleroderma?

Scleroderma can lead to scarring of the skin, joints, and other internal organs. The following are the most common symptoms of scleroderma. However, each individual may experience symptoms differently. Symptoms may include:

  • Thickening and swelling of the tips of the fingers
  • Pale and tingly fingers that may become numb when exposed to cold or when emotionally upset (called Raynaud's phenomenon)
  • Joint pain
  • Taut, shiny, darker skin on large areas such as the face, that may hinder movement
  • Appearance of spider veins
  • Calcium bumps on the fingers or other bony areas
  • Grating noise as inflamed tissues move
  • Frozen (immobile) fingers, wrists, or elbows due to scarring of the skin
  • Sores on fingertips and knuckles
  • Scarring of the esophagus, leading to heartburn and difficulty swallowing
  • Scarring of the lungs, leading to shortness of breath
  • Heart failure and abnormal heart rhythms
  • Kidney disease

The symptoms of scleroderma may resemble other medical conditions or problems. Always consult your doctor for a diagnosis.

How is scleroderma diagnosed?

In addition to a complete medical history and physical examination, a diagnosis of scleroderma is usually based on the changes in the skin and internal organs. An antibody test may help distinguish the type of scleroderma present.

Treatment for scleroderma

Specific treatment for scleroderma will be determined by your doctor based on:

  • Your age, overall health, and medical history
  • Extent of the condition
  • Your tolerance for specific medications, procedures, and therapies
  • Expectation for the course of the disease
  • Your opinion or preference

Treatment may include:

  • Nonsteroidal anti-inflammatory medications or corticosteroids (to relieve pain)
  • Immunosuppressive medications, such as penicillamine (to slow the skin thickening process and delay damage to internal organs)
  • Treating specific symptoms, such as heartburn and Raynaud's phenomenon
  • Physical therapy and exercise (to maintain muscle strength)

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