Limited Scleroderma (CREST Syndrome)

What is Limited Scleroderma?

Limited Scleroderma, or CREST syndrome, is a disease where scar tissue forms on finite portions of skin””mainly the hands and the face. This scar tissue is caused by an overproduction of collagen, which can build up and impair dexterity and different bodily systems. While diffuse scleroderm can affect internal organs, limited scleroderma is much less severe in its symptoms.

The cause of limited scleroderma is unknown; however, hereditary does play a role. The disease is seen more often in females than males, and it is seen more often in certain races, like those of African descent. Lastly, if exposed to certain toxins, some people with inadequate immune systems can acquire CREST syndrome.

What are the Symptoms of Limited Scleroderma?

Limited scleroderma is also known as CREST syndrome because the “CREST” acronym stands for the five main signs of the disease.

Those with the condition usually have calcium deposits on the skin, Raynald’s syndrome””which can restrict blood flow in the extremities””a weak or impaired esophagus, sclerodactyly””or epidermis tautness or fibrosis on toes and fingers, and dilated blood vessels that cause redness. Some complications of the disease include severe inflammation, blood vessel problems, heart problems, and lung problems.

Limited Scleroderma Causes

There are many causes of limited scleroderma. The main cause is thought to be the immune system acting abnormally and creating too much collagen. This excess collagen causes problems in many of the patient’s organs. However, it is believed that there are many other factors that can lead to patients developing this condition. There may be a genetic factor present, as those who have a family member who has an autoimmune disease are more likely to have it. Another risk factor is being female; more women develop this condition. Native Americans and blacks are also more likely to get it than white people.

There may also be an environmental aspect as well. People who have been exposed to toxins like benzene, polyvinyl chloride, trichloroethylene and silica are more likely to get this disease. These toxins are believed to trigger limited scleroderma to develop in people who are genetically predisposed to it. Another risk factor is age. This condition is most common in patients who are between 30 and 50 years old.

How is Limited Scleroderma Treated?

A plan might include one or more of the following treatments:

  • Antibiotics
  • Bandaging
  • Antacids
  • Blood pressure medications
  • Immunosuppresants
  • Physical Therapy
  • Occupational Therapy
  • Surgery
  • Amputation (if ulcers turn into severe gangrenes)

The direction of medical care is determined by which symptoms are most bothersome for a patient. For instance, if limited scleroderma is causing symptoms related to Raynaud’s syndrome, then blood-pressure lowering drugs may be taken to improve the circulation of blood in toes and fingers.

If a person has too much scar tissue, topical antibiotics, bandages, and drugs that suppress the inflammation can be used. If a person has enlarged capillaries, then laser surgery is recommended to eliminate those cosmetic blemishes on the skin. Because limited scleroderma can cause scar tissue on the hands, it is also important for patients to go to occupational therapy to keep those muscles strong, maintain normal range-of-motion, and break up thickened tissue. Lifestyle changes, proper skin care, and a well-balanced diet can also improve a patient’s symptoms.

Limited Scleroderma Prevention

Because this autoimmune condition is believed to have a large genetic component, there is currently no way to prevent it from occurring. There are treatments, however, that can prevent it from getting worse. Many patients need to manage their pain as well as take medications that will limit the damage that this syndrome does to their organs and tissues. Topical antibiotics can stop skin infections, and medications can lower the high blood pressure that is often caused by limited scleroderma. One of the preventative measures taken by patients who have serious cases of this condition is to take immune-suppressing drugs. This can prevent the patient’s immune system from attacking the body with too much collagen and slowing the progression of this condition. It can also prevent the damage to the lungs that limited scleroderma patients often experience.

Resources
Last Reviewed:
October 06, 2016
Last Updated:
March 12, 2018