Scleroderma is a connective-tissue disease that causes a progressive build up of tough scar-like tissue in the skin and internal organs. The term scleroderma is derived from the Greek words, skleros, meaning "hard," and derma meaning "skin." An individual with scleroderma may develop either a localized or systemic form of the disease. Localized scleroderma usually affects only the skin on the hands and face. Systemic scleroderma, however, affects the connective tissue in many parts of the body, including the skin, the esophagus, gastrointestinal tract, lungs, kidneys, heart, and other internal organs. It is unusual for localized scleroderma to progress to the systemic form. According to the Scleroderma Foundation, an estimated 300,000 people in the United States have the condition.

Symptoms of scleroderma may include one or more of the following:

• Raynaud's phenomenon (abnormal sensitivity to cold in the hands or feet)
• Swelling of the fingers, hands, forearms, feet, lower legs, and face
• Thickening, hardening, and discoloration of the skin
• Ulcers or lesions on fingers, face, tongue, and inner lining of the cheek
• Joint pain, swelling, and stiffness (especially in the fingers and knees)
• Carpal tunnel syndrome
• Shortness of breath, cough
• Dry eyes
• Sexual dysfunction
• Digestive and gastrointestinal problems including difficulty swallowing, bloating, and abdominal pain

Five particular symptoms occasionally occur together and are clinically recognized as a variation of scleroderma called CREST syndrome. The term CREST stands for Calcinosis (painful calcium deposits under the skin), Raynaud's phenomenon (abnormal 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 (lesions on the hands, palms, forearms, face, and lips).

Scleroderma results from an overproduction of collagen, the primary connective tissue protein in the body. Scientists believe that the immune system mistakenly attacks the patient's own cells (an autoimmune response), producing too many white blood cells and other factors that cause a damaging inflammatory response and an overproduction of collagen, primarily in the skin. Researchers are not clear why this autoimmune response occurs, but they suggest that both genetic and environmental factors play a role in the development of the disease. For example, scleroderma has been associated with a number of industrial and pharmaceutical chemicals including:

• Silica dust
• Some plastic materials, such as epoxy resins and vinyl chloride
• Aromatic hydrocarbons
• L-tryptophan (synthetic supplement)
• Rapeseed oil

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

• Gender -- approximately three times more women than men develop the disease.
• Age -- scleroderma is most common among individuals 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

An individual with symptoms of scleroderma will most likely require consultations with both a rheumatologist (arthritis specialist) and a dermatologist (skin specialist). The doctor will conduct 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 conduct the following procedures to diagnose the disease:

• Blood tests -- may detect elevated levels of antibodies found in the majority of individuals with scleroderma.
• Skin biopsy -- samples of skin may be taken to identify abnormalities suggestive of scleroderma.
• 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. This is because many individuals with scleroderma experience symptoms of other connective-tissue diseases, such as rheumatoid arthritis, lupus, and polymyositis. When these conditions overlap, it is called mixed connective-tissue disease.

It is important for an individual with scleroderma to avoid developing infections, so the physician will administer the following:

• Pneumococcal pneumonia vaccine
• Annual flu vaccine

There is no cure for scleroderma. While medications are often used to treat the symptoms of the disease, they are not always effective, and many have significant side effects. There are, however, less toxic ways to treat the symptoms of scleroderma, such as lifestyle and dietary changes and the addition of dietary supplements, which make living with the disease easier.

Lifestyle

While lifestyle adjustments will not stop the progression of scleroderma, these simple measures may enhance an individual's quality of life:

• Eating small, frequent meals may reduce gastrointestinal problems.
• Maintaining an active lifestyle may preserve muscle.
• Avoiding smoke, exposure to cold, and stress may prevent blood vessel spasms that diminish circulation.
• Applying soothing skin creams to affected areas may reduce pain, swelling, and stiffness.

Medications

Localized scleroderma often is treated with topical therapies such as moisturizers or topical corticosteroids. Oral medications 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, promote gastrointestinal function, preserve kidney function, and control high blood pressure. Some medications a physician may prescribe for scleroderma include:

• Penicillamine -- can reduce skin thickening and delay spread of damage to internal organs. However, it has relatively high incidence of adverse reactions.
• Pentoxifylline -- may improve circulation
• Immunosuppressant medications (such as cyclophosphamide) -- often used for patients with life-threatening form of the disease. These are potent medications that damage cells' genetic information. These medications may cause significant side effects.
• Anti-inflammatory medications (such as aspirin, ibuprofen, and corticosteroids) -- may reduce inflammation and swelling
• Calcium-channel blockers (such as nifedipine and diltiazem) -- often used to treat Raynaud's phenomenon
• Antibiotics -- prevent overgrowth of bacteria in the intestines and improve digestive processes
• ACE inhibitors (such as captopril) -- may improve hypertension, prevent kidney damage, and dilate blood vessels

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. Individuals with scleroderma tend to have deficiencies in many vitamins and minerals. 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.

Following these nutritional tips may help reduce symptoms:

• Try to eliminate potential food allergens, including dairy, wheat (gluten), corn, preservatives, and food additives. Your health care provider may want to test for food sensitivities.
• 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. Quality protein sources, such as organic meat and eggs, whey, and vegetable protein shakes, should be used as part of balanced program aimed at gaining muscle mass and preventing wasting.
• 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 coffee and other stimulants, alcohol, and tobacco.
• Drink 6 - 8 glasses of filtered water daily.
• Exercise at least 30 minutes daily, five days a week.

You may address nutritional deficiencies with the following supplements:

• A multivitamin daily, containing the antioxidant vitamins A, C, E, the B-complex vitamins, and trace minerals such as magnesium, calcium, zinc and selenium.
• Omega-3 fatty acids, such as fish oil, 1 - 2 capsules or 1 - 3 tablespoonfuls oil, one to three times daily, to help decrease inflammation and help with immunity. Cold-water fish, such as salmon or halibut, are good sources, but are not substitutes for supplementation.
• Vitamin C, 500 - 1000 mg, one to three times daily, as an antioxidant and for immune support.
• L-glutamine, 500 - 1000 mg three times daily, for support of gastrointestinal health and immunity.
• Dihydroepiandosterone (DHEA), start at 5 mg three times a day and work up to 100 mg daily for 7 - 12 months, for hormonal support. It is recommended to use DHEA under the supervision of a qualified health care professional. If adverse effects develop, discontinue use.
• Probiotic supplement (containing Lactobacillus acidophilus), 5 - 10 billion CFUs (colony forming units) a day, when needed for maintenance of gastrointestinal and immune health. You should refrigerate your probiotic supplements for best results.
• Grapefruit seed extract (Citrus paradisi), 100 mg capsule or 5 - 10 drops (in favorite beverage) three times daily when needed, for antibacterial, antifungal, and antiviral activity, and for immunity.
• Resveratrol (from red wine), 50 - 200 mg daily, to help decrease inflammation and for antioxidant effects.
• Coenzyme Q10, 100 - 200 mg at bedtime, for antioxidant and immune activity.
• Melatonin, 2 - 6 mg at bedtime as needed, for immune support and sleep.

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.

• Olive leaf (Olea europaea) standardized extract, 250 - 500 mg one to three times daily, for immune effects.
• Turmeric (Curcuma longa) standardized extract, 300 mg three times a day, for pain and inflammation.
• Milk thistle (Silybum marianum) seed standardized extract, 80 - 160 mg two to three times daily, for detoxification and antioxidant support.
• Rhodiola (Rhodiola rosea) standardized extract, 100 - 600 mg daily, for antioxidant, anti-stress, and immune activity.
• Bromelain (Ananus comosus) standardized, 40 mg three times daily, for pain and inflammation.
• Gotu kola (Centella asiatica) standardized extract, 50 - 250 mg two to three times daily, for blood vessel health and circulation.

Acupuncture

A few studies of patients with systemic scleroderma indicate that acupuncture may improve circulation in the hands and fingers, mend fingertip ulcers, and possibly reduce the formation of fibrous tissue. Acupuncture may also be effective for pain.

Massage and Physical Therapy

Research suggests that massage may be useful in improving circulation and preventing muscle distortion. More research is needed in this area to determine whether massage is truly an effective complementary therapy for scleroderma.

Mind-Body Medicine

Biofeedback appears to successfully control the temperature in the hands and feet of those with Raynaud's phenomenon, a symptom often found in those with scleroderma. Other mind-body techniques such as counseling, meditation, and emotional freedom technique (EFT) can be very useful.

Prognosis and Complications

Possible complications that may result from scleroderma include the following:

• Joint damage
• Damage to smooth muscles in the gastrointestinal tract
• Malnutrition
• Formation of fibrous tissue in the heart muscle; may lead to permanent damage and degradation
• Kidney damage and failure
• Formation of fibrous tissue in the thyroid gland

The prognosis for those with scleroderma is highly variable and depends primarily on the form of the disease. For example:

• The CREST syndrome tends to progress slowly and remains relatively benign for decades. 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 at the onset of 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 course of the disease, prognosis is generally poor.
• Spontaneous remissions from scleroderma have also been reported, with symptoms of the disease resolving in the reverse order that they appeared.

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