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Crest Syndrome - Symptoms & Treatment


Crest syndrome is a member of the heterogeneous group of sclerodermas, and its name is an acronym for the cardinal clinical features of the syndrome. Crest syndrome is a subset of scleroderma, a disorder that leads to thickening, hardening and tightening of your skin and connective tissue. Because it's so pervasive and varied, connective tissue can give rise to hundreds of complex and puzzling disorders. These forms of scleroderma are rare in children. Crest is an acronym for the clinical features that are seen in a patient with this disease.Women experience this disorder four times more often than men and it is rarely found in children. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin. Most patients with Crest syndrome have the limited cutaneous form of scleroderma. Limited cutaneous scleroderma generally causes thickening of the skin distal (toward the fingers) to the elbows but may include the face.

Crest syndrome usually affects people 30 to 50 years old. Females have a greater incidence of scleroderma than males. This difference appears greater during childbearing years. In people with Crest syndrome, the immune system appears to stimulate cells called fibroblasts to produce excess amounts of collagen. Scleroderma also contributes to the accumulation of scar tissue in the lungs, which can have a negative effect on breathing during exercise. Telangiectasia can be a symptom of scleroderma or other systemic diseases. In the skin, ulceration, calcification, and changes in pigmentation may occur. Risk factors are occupational exposure to silica dust and polyvinyl chloride. The association of the two conditions is clinically and etiologically important. Clinicians must be aware of this association, sincethe clinical features of Crest syndrome may be mild and may be thought to be complications of the underlying liver disease. Conventional medical treatments may help relieve the symptoms of Crest syndrome but they do not address the root of the problem.

Causes of Crest syndrome

The common causes and risk factor's of Crest syndrome include the following:

  • The exect cause of Crest syndrome is unknown.
  • Genetic makeup.
  • Exposure to viral infections and some environmental toxins may trigger scleroderma in people who are genetically predisposed to develop the disease.
  • Environmental factor's.
  • Exposure to toxins.
  • Genetic factors.

Symptoms of Crest syndrome

Some sign and symptoms related to Crest syndrome are as follows:
  • Esophageal dysfunction.
  • Pain, stiffness, and swelling of fingers and joints.
  • Aches and pains in the joints.
  • Heartburn.
  • Raynaud's phenomenon.
  • Hair loss.
  • eye burning, itching and discharge.
  • Difficulty swallowing and shortness of breath.

Treatment of Crest syndrome

Here is list of the methods for treating Crest syndrome:

  • Tamoxifen has been studied for use in patients with scleroderma and Creast syndrome, but it was not shown to be efficacious.
  • Certain lifestyle changes can help reduce problems with swallowing and especially with acid reflux.
  • Dilated capillaries can be hidden with makeup or reduced or eliminated with laser therapy.
  • Calcium channel blockers are the mainstay of medical therapy for Raynaud phenomenon.
  • Treatment with oral corticosteroids is not usually considered effective, but, according to Hazen et al, intralesional corticosteroid therapy has been associated with improvement of calcinosis.
  • Very large or painful calcium deposits are sometimes surgically removed.

 

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