CREST Syndrome

Some people have a type of scleroderma called CREST syndrome (or limited scleroderma). Unlike other types, which only affect the arms, legs, and face, this kind can involve your digestive tract. While less common, it can also lead to problems with your heart and lungs.

What is Crest Syndrome?

  • 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.

Causes of Crest Syndrome

  • Doctors don’t know the exact cause, but they do know what happens when you have scleroderma.

  • The problem is with your immune system. For some reason, it prompts your body to make too much of the protein collagen.

  • The result is thicker, less flexible skin and other tissues in your body.

Symptoms of Crest Syndrome

  • Swelling, stiffness, or pain in the fingers, toes, hands, feet, or face

  • Puffy skin

  • Discolored skin

  • Raynaud’s phenomenon, or fingers and toes that react strongly to cold and may look white and be painful.

  • Telangiectasias red spots on the fingers, palms, face, lips, or tongue that happen when tiny blood vessels are widened

  • Ulcers or sores on fingertips, knuckles, or elbows

  • Fatigue or feeling tired

  • Tight skin across your face

  • Stiff, painful joints

Diagnosis of Crest Syndrome

  • If you think you have scleroderma, tell your doctor what symptoms you’ve noticed.

  • In order to make a diagnosis, they’ll ask you about your family’s health history, look for changes in how thick your skin is, and do some tests.

  • They may also look at your finger under a microscope to check for changes in tiny blood vessels.

  • These start to vanish early on in scleroderma.

  • They’ll likely take a blood sample and send it to the lab to see if your immune system is in overdrive.

  • Your doctor may also take a small sample of your skin for testing.

  • That’s called a biopsy.

  • It’s helpful because scleroderma can be hard to diagnose.

Treatment of Crest Syndrome

  • Skin: Try using moisturizers or corticosteroid creams.

  • Joint or other pain: Nonsteroidal anti-inflammatory drugs (NSAIDs), like aspirin, naproxen, and ibuprofen, may help. Your doctor may prescribe corticosteroid pills, such as prednisone, to lower inflammation.

  • Raynaud’s phenomenon: This strong reaction to cold and stress in your hands and feet can cause them to turn white and ache. Your doctor may give you medicines to relax and open the blood vessels. You might hear them referred to as vasodilators.

  • Stomach trouble: You can take medications to lower stomach acids and control heartburn. Your doctor may prescribe medicines that help move food through your stomach and intestines. They may also try antibiotics or a special diet.

  • Kidney problems: You might take prescription drugs to control your blood pressure. One kind often used is called an angiotensin-converting enzyme (ACE) inhibitor.

  • Systemic scleroderma: Your doctor may give you drugs that suppress your immune system, like mycophenolate, cyclophosphamide, and methotrexate. Although these aren’t approved for scleroderma, doctors often prescribe them to help.

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