Introduction:

Scleroderma is a type of connective-tissue disease that causes skin and sometimes internal organs to become hard and tight. In fact, the word scleroderma actually means "hard skin." Scleroderma occurs when the body produces too much collagen, the protein that makes up connective tissues. You can have localized scleroderma, which usually only affects the skin on the hands and face, or systemic scleroderma, which affects connective tissue in many parts of your body, including internal organs. Scleroderma is considered an autoimmune disease, meaning that the immune system mistakenly attacks the body's own tissues. According to the Scleroderma Foundation, about 300,000 people in the United States have the condition.

Signs and Symptoms:

Symptoms of scleroderma may include the following:

Localized scleroderma

• Morphea scleroderma -- oval patches of thick skin that are white in the middle and purple around the edges. Patches usually appear on the chest, back, and stomach, but can also be on arms and legs.
• Linear scleroderma -- streaks of hardened skin appearing on arms, legs, or forehead.

Systemic scleroderma

• Diffuse cutaneous systemic sclerosis -- hardening of skin on fingers, hands, arms, legs, face, neck, and trunk. This type of scleroderma is usually symmetrical (meaning if the left arm is affected, the right arm will be, too). It also can affect internal organs, including heart, lungs, kidneys, and esophagus.
• Limited cutaneous systemic sclerosis -- affects skin on fingers, lower arms and legs, face, and neck. People with this type of scleroderma often have CREST syndrome. The term CREST stands for Calcinosis (painful calcium deposits under the skin), Raynaud's phenomenon (extreme sensitivity to cold in the hands and feet), Esophageal dysfunction (problems with swallowing caused by internal scarring), Sclerodactyly (tightening of the skin on the fingers or toes) and Telangiectasia (swollen blood vessels on the hands, palms, forearms, face, and lips).
• Sine scleroderma -- affects internal organs, but not skin

Causes:

Scleroderma occurs when the immune system mistakenly attacks the body's own tissues, causing inflammation and an overproduction of collagen. Too much collagen causes the skin, and sometimes the internal organs to become hard and tight. Researchers aren't sure what triggers this autoimmune response, but both genetics and environment may play a role.

Risk Factors:

The following factors may increase a person's risk for scleroderma:

• Gender -- About three times more women than men develop the disease.
• Age -- scleroderma is most common among people between the ages of 20 - 50, and is relatively rare in children.
• Race and ethnicity -- young women of African ancestry and native Americans of the Choctaw tribe of Oklahoma have a high prevalence of the disease.

Diagnosis:

It isn't always easy to diagnose scleroderma. You may need to see both a rheumatologist (arthritis specialist) and a dermatologist (skin specialist). The doctor will perform a physical examination in which he or she may feel the skin, checking for thickened and hardened areas, and may also press affected tendons and joints. The doctor may also perform the following procedures:

• Blood tests -- may detect higher levels of antibodies produced by the immune system.
• Skin biopsy -- may detect skin abnormalities.
• Chest X ray or pulmonary function test -- may detect lung damage or altered lung capacity.
• MRI or CT scan -- often detect early signs of damage to the muscles and internal organs.

Diagnosing scleroderma may be difficult, particularly in the early stages of the disease. In part, scleroderma is difficult to diagnose because the early symptoms are like those of other connective-tissue diseases, such as rheumatoid arthritis, lupus, and polymyositis. When these conditions overlap, it is called mixed connective-tissue disease.

Preventive Care:

Although no one knows how to prevent scleroderma, you can take steps to avoid developing infections when you have scleroderma. Your doctor may recommend:

• Pneumococcal pneumonia vaccine
• Annual flu vaccine

Treatment:

There is no cure for scleroderma. Medication can treat symptoms and may help prevent complications. Lifestyle and dietary changes may make living with the disease easier.

Lifestyle

These simple steps may help improve quality of life:

• Eating small, frequent meals may reduce heartburn or gas.
• Exercise helps keep skin and joints flexible.
• Don't smoke, because nicotine contracts blood vessels, which makes scleroderma worse.
• Avoid exposure to cold and stress, which can also affect circulation.
• Use soothing skin creams to reduce pain, swelling, and stiffness.

Medications

Localized scleroderma often is treated with topical moisturizers or corticosteroids. Oral medications such as minocycline (Minocin or Dynacin) may also be used to halt the progression of localized scleroderma if it involves a large area of the body, such as an entire arm or leg.

Systemic scleroderma may be treated with medications that improve circulation, reduce heartburn, preserve kidney function, and control high blood pressure. Some medications a physician may prescribe for scleroderma include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) -- to relieve joint pain and inflammation
• Medications to promote better circulation -- can help prevent high blood pressure and reduce symptoms of scleroderma. They include:
  • Calcium channel blockers
  • Angiotensin II receptor blockers
  • Angiotensin-converting enzyme (ACE) inhibitors
  • Alpha blockers
  • Aspirin
• Disease-modifying anti-rheumatic drugs (DMARDs) -- slow the progression of the disease. They include:
  • Hydroxychloroquinine (Plaquenil)
  • Methotrexate (Rheumatrex)
  • Sulfasalazine (Azulfidine)
• Immunosuppressants -- suppress an overactive immune system. These drugs can have serious side effects including kidney damage and increased risk of infection. they include:
  • Azathioprine (Imuran)
  • Cyclophosphamide (Cytoxan)
  • Cyclosporine (Neoral)
• Antacids -- to reduce heartburn when there is damage to the esophagus

Surgery and Other Procedures

When symptoms of scleroderma become very severe, physicians may recommend the following procedures:

• Surgery to repair damage to the stomach or intestinal walls
• Amputation of severely diseased and infected fingers or toes
• Kidney, heart, or lung transplantation (in rare cases)

Nutrition and Dietary Supplements

A comprehensive treatment plan for scleroderma may include a range of complementary and alternative therapies. People with scleroderma may not get enough vitamins and minerals in their diet, especially if there is damage to their gastrointestinal system. Ask your team of health care providers about the best ways to incorporate these therapies into your overall treatment plan. Always tell your health care provider about the herbs and supplements you are using or considering using.

These general nutritional tips are important for anyone with a chronic disease:

• Eat antioxidant foods, including fruits (such as blueberries, cherries, and tomatoes), and vegetables (such as squash and bell peppers).
• Avoid refined foods such as white breads, pastas, and especially sugar.
• Eat fewer red meats and more lean meats, cold-water fish, tofu (soy, if no allergy) or beans for protein.
• Use healthy oils, such as olive oil or vegetable oil.
• Reduce or eliminate trans-fatty acids, found in commercially baked goods such as cookies, crackers, cakes, French fries, onion rings, donuts, processed foods, and margarine.
• Avoid caffeine and other stimulants, alcohol, and tobacco.
• Drink 6 - 8 glasses of filtered water daily.
• Exercise at least 30 minutes daily, five days a week.
• Your doctor may also recommend taking a multivitamin daily, containing the antioxidant vitamins A, C, E, the B-complex vitamins, and trace minerals such as magnesium, calcium, zinc and selenium.

These supplements may help reduce some symptoms:

• Omega-3 fatty acids, such as fish oil, 1 - 2 capsules or 1 - 3 tablespoonfuls oil, one to three times daily. Omega-3 fatty acids help improve circulation, and a few studies suggest they may reduce symptoms of Raynaud's phenomenon and improve tolerance to cold. Cold-water fish, such as salmon or halibut, are good sources.
• Probiotic supplement (containing Lactobacillus acidophilus), 5 - 10 billion CFUs (colony forming units) a day. Probiotics, or "friendly" bacteria, can help maintain gastrointestinal health. That is especially important when you are taking antibiotics. You should refrigerate your probiotic supplements for best results.

Herbs

Herbs are generally a safe way to strengthen and tone the body's systems. As with any therapy, you should work with your health care provider to get your problem diagnosed before starting any treatment. You may use herbs as dried extracts (capsules, powders, teas), glycerites (glycerine extracts), or tinctures (alcohol extracts). Unless otherwise indicated, you should make teas with 1 tsp. herb per cup of hot water. Steep covered 5 - 10 minutes for leaf or flowers, and 10 - 20 minutes for roots. Drink 2 - 4 cups per day. You may use tinctures alone or in combination as noted.

Very few studies have been done using these herbs to treat scleroderma. Ask your doctor before adding any of these herbs to your treatment plan.

• Turmeric (Curcuma longa) standardized extract, 300 mg three times a day. Lab studies show that turmeric reduces inflammation. It may also help relieve pain, although more studies are needed to tell whether it has any specific effect on scleroderma.
• Bromelain (Ananus comosus) standardized, 40 mg three times daily. Although not a specific treatment for scleroderma, bromelain also helps reduce pain and inflammation.
• Gotu kola (Centella asiatica) standardized extract, 50 - 250 mg two to three times daily, for blood vessel health and circulation. Some purified extracts of gotu kola seem to reduce symptoms of scleroderma. More research is needed to know whether it truly has any benefit.

Acupuncture

A few studies suggest that acupuncture may improve circulation in the hands and fingers, help heal fingertip ulcers, and possibly reduce the formation of fibrous tissue in people with scleroderma. Acupuncture may also lessen pain.

Massage and Physical Therapy

Research suggests that massage may help improve circulation and prevent muscle distortion. More research is needed to determine whether massage is truly an effective therapy for scleroderma.

Mind-Body Medicine

Biofeedback may help people with scleroderma better control the temperature in their hands and feet. Other mind-body techniques such as counseling, meditation, and emotional freedom technique (EFT) can be very useful.

Other Considerations:

Prognosis and Complications

Possible complications include the following:

• Joint damage
• Damage to smooth muscles in the gastrointestinal tract, leading to malnutrition
• Scarring of heart muscle; may lead to permanent damage
• Kidney damage and failure

The prognosis for people with scleroderma depends primarily on the form of the disease. For example:

• The CREST syndrome tends to progress slowly. The 10-year survival rate for those with CREST is 75%.
• Those with localized scleroderma also have a 10-year survival rate of 75%.
• Systemic scleroderma tends to progress faster in men and in those who are older when they get the disease. The 10-year survival rate for those with systemic scleroderma is 55%. If damage spreads to the heart, lung, or kidneys early in the disease, prognosis is generally poor.
• Some people may experience spontaneous remission from scleroderma, with symptoms of the disease disappearing in the reverse order that they appeared.