CREST syndrome , also known as limited scleroderma, is a widespread connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs .
The symptoms involved in CREST syndrome are associated with the generalized form of the disease systemic sclerosis (scleroderma).
+ Tight, hardened skin. In limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and neck. Skin can look shiny from being pulled taut over underlying bone. It may become difficult to bend your fingers or to open your mouth.
+ Raynaud’s phenomena. This condition occurs when small blood vessels in your fingers and toes spasm in response to cold or emotional stress, blocking the flow of blood. In most people, the skin turns white before becoming blue, cold and numb.
When circulation improves, the skin usually reddens and might throb or tingle. Raynaud’s phenomena is often the first sign of limited scleroderma, but many people who have Raynaud’s never develop scleroderma.
+ Red spots or lines on skin. The swelling of tiny blood vessels near the skin’s surface cause these small red spots or lines (telangiectasias). Not painful, they occur primarily on the hands and face.
Bumps under the skin. Limited scleroderma can cause tiny calcium deposits (calcinosis) to develop under your skin, mainly on your elbows, knees and fingers. You can see and feel these deposits, which sometimes are tender or become infected.
Swallowing difficulties. Limited scleroderma commonly causes problems with the tube that connects the mouth and stomach (esophagus). Poor functioning of the muscles in the upper and lower esophagus can make swallowing difficult and allow stomach acids to back up into the esophagus, leading to heartburn, inflammation and scarring of esophageal tissues.
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+ Blood exams: A blood sample can be tested for antibodies (ANA and anticentromere) that are frequently found in the blood of people with limited scleroderma. This isn’t a definitive test because not everyone with limited scleroderma has these antibodies.
+ Skin Biopsy: Sometimes doctors take a small sample of skin that is then examined under a microscope in a laboratory. Biopsies can be helpful, but they can’t definitively diagnose limited scleroderma either.
- Additional tests: Along with a blood test and skin biopsy, additional tests to identify lung, heart or gastrointestinal complications may also be conducted. The diagnosis of calcinosis (suspected based on finding the nodules) is confirmed with imaging studies. Multiple imaging studies are available to evaluate esophageal motility. The least invasive evaluation involves radiologic barium studies.
Unfortunately, CREST syndrome has no known cure. Treatment generally focuses on relieving symptoms and preventing complications:
Calcinosis: No treatment seems to be available to prevent or eliminate calcinosis. Large or painful calcium deposits sometimes need to be surgically removed, and amputation of fingertips may be necessary if the skin ulcers progress to gangrene. One or a combination of the following treatments may be tried on a case-by-case basis; however, larger studies are needed to prove efficacy.
- Corticoids oral or injected inside the lesion (intralesional)
- Aluminum hydroxide
- Intravenous immunoglobulin therapy
Raynaud phenomenon: The following treatment is suggested for the treatment of people with Raynaud phenomenon:
- Reduce and remove risk factors and possible triggers such as smoking, avoid a medication known as beta-blockers, and avoid any other remediable underlying cause (eg, use of vibratory equipment)
- Keep hands and body warm
- Take a medication that are long-acting formulations of calcium channel blockers
- Use topical nitroglycerin paste if needed.
Esophageal dysmotility: The treatment of esophageal dysmotility and gastroesophageal reflux in people with scleroderma is the same as in patients without scleroderma. Treatment involves behavior changes, medication (H2 blockers), and a surgical procedure (esophageal dilatation) in more severe cases. Esophageal dilatation can help when severe feeding difficulty or regurgitation occur due to an esophageal narrowing.
Sclerodactyly: Treatments include medication (corticosteroids, nonsteroidal anti-inflammatory drugs, D-penicillamine, IFN-gamma, cyclosporine, and cytostatic drugs). Skin involvement in CREST syndrome typically is not severe; therefore, treatment is not need in many cases.
Telangiectasia: Pulsed-dye laser treatment seems effective for the treatment the telangiectasia in the face, but this has not been specifically studied in CREST patients. Treatment may include estrogen-progesterone or desmopressin, laser ablation, or sclerotherapy.
Other treatment may include stretching exercises for the finger joints, and facial exercises that may help keep the face and mouth flexible, under a physical therapist supervision. If CREST syndrome is making it difficult to do daily tasks, an occupational therapist can help individuals learn new ways of doing things. For example, special toothbrushes and flossing devices can make it easier to care for the teeth. Surgery may be necessary for some affected individuals.
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