CREST is a type of collagen vascular disease. It is believed to be an autoimmune condition. CREST is a type of scleroderma.
There are two types of scleroderma -- localized and systemic -- and CREST is considered a mild form of systemic scleroderma. The prognosis for CREST is usually very good. Some people with scleroderma also have other collagen vascular disease, or a hybrid of two types (an "overlap" syndrome), so some evaluation is always needed.
The term CREST is an acronym derived from the five most prominent features:
C - Calcinosis - calcium deposits in the subcutaneous layer of the skin
R - Raynaud's phenomenon - extreme sensitivity to cold or stress
E - Esophageal dysfunction - swallowing problems caused by scarring
S - Sclerodactyly - tightening of the skin confined to fingers and toes
T - Telangiectasia - red spots on the hands, palms, forearms, face and lips
The accumulation of calcium below the outer layer of the skin. Many parts of the body can be affected such as: fingers, arms, feet & knees. Pressure on the skin from these deposits can cause severe pain, ulcerations and infection if the calcium protrudes.
Infection is treated with antibiotics administered orally or intravenously. In some cases, hospitalization is required when surgery is the only way to stop the infection.
A vascular disturbance affecting the extremities, primarily the hands. Spasms of the small blood vessels (capillaries) cause color changes varying from white to blue to red. Exposure to cold or emotional stress can intensify the problem; and there may be pain, tingling, numbness or a burning sensation. Care of the hands is most important as ulcerations and infections may occur if they are bruised or cut. Extreme cleanliness is essential when there are ulcerations. Mittens should be worn to protect the hands from cold, even when using the refrigerator or freezer. Patients are advised to protect the entire body from cold. There are many drugs and treatments used with varying success to alleviate symptoms including: methyldopa (Aldomet), prazosin (Minipress), nifedipine (Procardia), verapamil (Calan), pentoxifylline (Trental), nitroglycerine paste, occlusive dressings, such as Duoderm, paraffin baths and biofeedback therapy.
The loss of normal motility of the lower esophagus. This causes difficulty swallowing. A common complaint is acid reflux, with resulting heartburn. Should the esophagus become too restricted, dilation is usually the treatment. To aid in overcoming reflux action, several small meals a day should be taken combined with small amounts of liquids. Do not eat at bedtime. It is advised to sit upright one to two hours after eating. Blocks may be used to raise the head of the bed six inches, and antacids may be prescribed for use an hour after meals and at bedtime. (Tagamet) cimetidine and (Zantac) ranitidine have also been prescribed for this condition. They are anti-ulcer drugs that help to protect the stomach and intestine linings from stomach acids by reducing the amount of acid released into the stomach.
A condition in which the skin of the fingers becomes taut, thin and shiny. Fingers and toes may not bend or may become fixed in a flexed or less functional position. For this problem, physical or occupational therapy may be a valuable aid. Prescription vitamin D pills and ointment have recently been found to be mildly helpful.
The appearance of capillaries near the surface of the skin. They will blanch on pressure. These capillaries become dilated and visible appearing chiefly on the face, lips, tongue, fingers and palms. Special cosmetics may be used to lessen visibility (Dermablend and Covermark, others).
There are no treatments proven effective enough to use in most cases, but promising experimental treatments are available for some of the more severe cases. Treatment with minocycline, oral Trental and calcium channel blockers are sometimes used. A recent advance is the use of Diovan (valsartan) which is helpful for the symptoms caused by Raynaud's phenomenon. If an ulcer develops it can be healed by topical nitroglycerin ointment applied to the borders of the ulcer twice daily.
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