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What is CREST Syndrome?

August 6, 2020
Published in: Primary Care, Rheumatology

Man rubbing a sore hand

Scleroderma is a rare disease, affecting an estimated 300,000 Americans—less than .1% of the country's population. CREST Syndrome, a subcategory of the disease, is rarer still. As a result, it is even more deserving of some time in the spotlight. The more we can raise awareness, the more accurately the condition can be diagnosed. And while it is a chronic disease with no known cure, it can be made more manageable with treatment.

What is Scleroderma?

Scleroderma is a term used to describe a rare group of autoimmune diseases. Like most autoimmune diseases, your body is unable to properly control its system, and in this case, it leads your body to make too much of the protein collagen. When this happens, it typically causes your skin to get thick and tight. The name scleroderma, comes from the Greek "sclero" meaning hard and "derma" meaning skin.

In most cases of scleroderma the symptoms result in hard, oval-shaped patches on the skin or lines or streaks on arms, legs, or face. Other symptoms might include puffy or swollen fingers and/or toes, painful or swollen joints, muscle weakness, dry eyes or mouth, swelling, shortness of breath, heartburn, diarrhea, or weight loss.

CREST Syndrome—a Category of Scleroderma

CREST syndrome is the name given to the specific type of scleroderma that affects your digestive tract and in rare cases, your heart and lungs. It gets its name from the specific symptoms of the condition:

  • Calcinosis: Painful lumps of calcium in the skin. These can happen on your fingers or other parts of your body.
  • Raynaud's phenomenon: White or cold skin on the hands and feet when you're cold or stressed. It's caused by blood flow problems.
  • Esophageal dysfunction: Problems swallowing and/or reflux. This is caused by scarring in the esophagus, the tube that runs from your mouth to your stomach.
  • Sclerodactyly: Tightness and thickening of finger or toe skin. It can make it hard to bend your fingers.
  • Telangiectasias: Red spots on the hands, palms, forearms, face, and lips. These are caused by widened blood vessels.

How is scleroderma treated and diagnosed?

Diagnosis of scleroderma or CREST syndrome will depend on the organs affected, but will generally involve either a rheumatologist or dermatologist. The condition is not contagious, nor is it cancerous, but can lead to further complications if its symptoms are not managed properly.

Treatment for scleroderma usually reflect the symptoms that it causes. With CREST syndrome, for example, in which issues with acid reflux are more common, heartburn medication may be issued. A doctor may prescribe NSAIDS, nonsteroidal anti-inflammatory drugs, to manage swelling or pain. Other treatments might include steroids, drugs that improve blood flow like ibuprofen or Aspirin, or blood pressure medication.

A doctor may also encourage exercise, skin treatment like light and laser therapy, physical therapy, occupational therapy, or in very severe cases where organ damage has occurred, an organ transplant.

As with any chronic condition, early detection and diagnosis is the most important faction in managing the disease. If you have questions about your health, start by contacting your primary care provider.