Medical scleroderma crest

by Nathan Wei, MD, FACP, FACR

Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.

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Scleroderma is an autoimmune disease involving the connective tissue.

Scleroderma is characterized by the formation of fibrosis in the skin and organs of the body. This leads to thickness of involved areas. Scleroderma is also referred to as systemic sclerosis.

The cause of scleroderma is not known. Researchers have found some evidence that genes are important factors, but the environment seems to also play a role. The disease is more frequent in females than in males.

Scleroderma can be classified in terms of the degree and location of the skin involvement. Accordingly, scleroderma has been categorized into two major groups, diffuse and limited.

The diffuse form of scleroderma is involves symmetric thickening of skin of the arms, legs, face, trunk (chest, back, abdomen, or flanks) which can rapidly progress to hardening after an early inflammatory phase. Organ disease can occur early on and be serious. Organs affected include the esophagus, bowels, lungs with scarring (fibrosis), heart, and kidneys. High blood pressure can be a serious consequence.

The limited form of scleroderma tends to be confined to the skin of the fingers and face. The skin changes and other features of disease tend to occur more slowly than in the diffuse form. Because a characteristic clinical pattern can occur in patients with the limited form of scleroderma, this form has taken another name which is composed of the first initials of the common components. Thus, this form is also called the CREST variant of scleroderma. This name represents the following features:

Calcinosis refers to the formation of tiny deposits of calcium in the skin. This is seen as hard whitish areas in the superficial skin, commonly overlying the elbows, knees, or fingers. These firm deposits can be tender, can become infected, or require surgical removal.

Raynaud's phenomenon refers to the spasm of the tiny artery vessels supplying blood to the fingers, toes, nose, tongue, or ears. These areas turns blue, white, then red after exposure to extremes of cold, or even sometimes with extremes of heat or emotional upset.

Esophagus disease in scleroderma is characterized by abnormal functioning muscle of the lower 2/3 of the esophagus. This can lead to a "lax" esophagus which allows stomach acid to reflux into the esophagus and cause heartburn, inflammation, and potentially scarring. This can eventually lead to difficulty in passing food from the mouth through the esophagus into the stomach. Symptoms of heartburn are treated aggressively in patients with scleroderma in order to prevent injury to the esophagus.

Sclerodactyly is localized thickening and tightness of the skin of the fingers or toes. This can give them a "shiny" and slightly puffy appearance. The tightness can cause severe limitation of motion of the fingers and toes. These skin changes generally progress much slower that those of patients with the diffuse form of scleroderma.

Telangiectasias are tiny red areas, frequently on the face, hands and in the mouth behind the lips. These areas blanch when they are pressed upon and represent dilated capillaries.

Patients can also have "overlap" illness with features of both CREST and the diffuse form of scleroderma. Some patients have overlaps of scleroderma and other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and polymyositis. When features of scleroderma are present along with features of polymyositis and systemic lupus erythematosus, the condition is referred to as mixed connective tissue disease (MCTD).

Finally, scleroderma skin changes can be localized. Morphea is scleroderma skin that is localized to a patchy area of the skin that becomes hardened and slightly pigmented. Sometimes morphea can cause multiple areas of damage in the skin. Linear scleroderma is scleroderma that is localized usually to a lower extremity, frequently presenting as a strip of hardening skin down the leg of a child.

The diagnosis of the scleroderma syndrome is based on the finding of the clinical features of the illnesses. Nearly all patients with scleroderma have blood tests which suggest autoimmunity, antinuclear antibodies (ANAs). A particular antibody, the anticentromere antibody, is found almost exclusively in the limited, or CREST, form of scleroderma. Anti-Scl 70 antibody is most often seen in patients with the diffuse form of scleroderma.

Other tests may be required. These may include upper and lower gastrointestinal studies to evaluate the bowels, chest x-rays, and lung function tests to examine the lungs, EKG and echocardiograms to evaluate the heart.

Aggressive treatment of elevations in blood pressure has been extremely important in prevent kidney failure.

Skin itching can be relieved with lotions (emollients), such as Eucerin and Lubriderm.

Mild Raynaud's phenomenon may require only hand warming and protection. Low dose aspirin is often added to prevent tiny blood clots in the fingers, especially in patients with a history of fingertip ulcerations. Moderate Raynaud's phenomenon can be helped by medications that open up the arteries, such as nifedipine (Procardia, Adalat) and nicardipine (Cardizem), or with topical nitroglycerin applied to the most affected digit. Severe Raynaud's phenomenon can require surgical procedures to interrupt the nerves of the finger that stimulate constriction of the blood vessels (digital sympathectomy). Ulcerations of the fingers can require topical or oral antibiotics.

Esophagus irritation and heartburn can be relieved with omeparazole (Prilosec), esomeprazole (Nexium), or lansoprazole (Prevacid). Antacids can also be helpful. Elevating the head of the bed can reduce backflow of acid into the esophagus that causes inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps.

Constipation, cramping and diarrhea is sometimes caused by bacteria that can be treated with tetracycline or erythromycin. Increased fluid intake and fiber intake are good general measures.

Irritated, itchy dry skin can be helped by lubricants, such as Lubriderm, Eucerin, or Bagbalm.

Telangiectasias, such as those on the face, can be treated with laser therapy.

Approximately 10 percent of patients with the CREST variant develop pulmonary hypertension. Abnormally elevated blood pressure of the arteries supplying the lungs is often treated with calcium antagonist medications, such as nifedipine, and blood thinning drugs (anticoagulation). More severe pulmonary hypertension can be helped by continuous intravenous infusion of prostacyclin (Iloprost). A drug taken by mouth, bosartan (Tracleer), is now available to treat severe pulmonary hypertension.

Additionally, medications are used to suppress immune system can be used. Medications used for this purpose include azathioprine, and methotrexate. Serious inflammation of the lungs (alveolitis) can require immune suppression with cyclophosphamide (Cytoxan) along with prednisone.

Recent data indicates that the critical period of organ risk is generally within the first three years of skin involvement. This means that patients can be reassured that their risk of organ-threatening complications is significantly less after three years of having skin symptoms.

Many researchers are investigating the roles of various cell messengers, called cytokines, in causing scleroderma.

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