Scleroderma is a group of diseases that causes skin and sometimes internal organs to become hard and tight. In fact, the word scleroderma actually means "hard skin." It happens when the body makes too much collagen, the protein that makes up connective tissues.
Localized scleroderma usually only affects the skin on the hands and face. Systemic scleroderma is more serious and 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. It is more common in women than men.
Signs and Symptoms:
Symptoms of scleroderma may include the following:
- Morphea scleroderma -- oval patches of thick skin that are white in the middle and purple around the edges. They usually appear on the chest, back, and stomach, but can also be on arms and legs.
- Linear scleroderma -- streaks of hardened skin that appear on arms, legs, or forehead.
- Diffuse cutaneous systemic sclerosis -- hardening of skin on fingers, hands, arms, legs, face, neck, and trunk. This type of scleroderma usually affects both sides of the body, meaning if you have problems with your left arm, you'll have problems with your right arm, 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, 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
- Telangiectasia, swollen blood vessels on the hands, palms, forearms, face, and lips
- Sine scleroderma -- affects internal organs, but not skin
Doctors believe scleroderma is caused by the immune system mistakenly attacking the body's own tissues. The immune system attack causes inflammation and triggers the body to make too much 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. Both genetics and environment may play a role.
These factors may increase the risk of scleroderma:
- Gender -- About three times more women than men get scleroderma.
- Age -- scleroderma is most common among people between the ages of 20 - 50. It is relatively rare in children.
- Race and ethnicity -- young women of African ancestry and native Americans of the Choctaw tribe of Oklahoma have high rates of the disease.
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 do a physical examination and feel your skin to check for thickened and hardened areas. The doctor may also press affected tendons and joints and do the following:
- Blood tests -- may find higher levels of antibodies made by the immune system.
- Skin biopsy -- may find skin problems.
- Chest X ray or pulmonary function test -- may find lung damage.
- MRI or CT scan -- often finds early signs of damage to the muscles and internal organs.
Many early scleroderma symptoms are like those of other connective-tissue diseases, such as rheumatoid arthritis, lupus, and polymyositis. When someone has more than one of these diseases, it is called mixed connective-tissue disease.
Although no one knows how to prevent scleroderma, you can take steps to avoid getting infections when you have scleroderma. Your doctor may recommend:
- Pneumonia (pneumococcal) vaccine
- Annual flu vaccine
There is no cure for scleroderma. Medications can treat symptoms and prevent complications. Making changes to your lifestyle and diet can make living with the disease easier.
These simple steps may help improve quality of life:
- Eat small, frequent meals to reduce heartburn or gas.
- Exercise to help keep skin and joints flexible.
- Don't smoke, because nicotine makes scleroderma worse.
- Avoid exposure to cold and stress, which can affect circulation.
- Use soothing skin creams to reduce pain, swelling, and stiffness.
Localized scleroderma often is treated with moisturizers or steroid creams. Oral medications such as minocycline (Minocin or Dynacin) may also be used to stop localized scleroderma from getting worse 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 doctor 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
- 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, doctors may recommend the following procedures:
- Surgery to fix damage to the stomach or intestinal walls
- Amputation of severely diseased and infected fingers or toes
- Kidney, heart, or lung transplants, in rare cases
Nutrition and Dietary Supplements
People with scleroderma may not get enough vitamins and minerals in their diet, especially if there is damage to their digestive system. Your doctor may suggest you take a supplement. Always tell your doctor about the herbs and supplements you are using or considering using.
These general nutritional tips are good for your overall health, especially if you have a long-lasting 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, and processed foods.
- Avoid caffeine, alcohol, and tobacco.
- Drink 6 - 8 glasses of filtered water daily.
- Exercise at least 30 minutes daily, 5 days a week.
Your doctor may recommend taking a multivitamin daily that has 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 blood flow, and a few studies suggest they may reduce symptoms of Raynaud's phenomenon and help people better tolerate cold temperatures. Cold-water fish, such as salmon or halibut, are good sources. Omega-3 supplements may increase your risk of bleeding. If you take blood-thinners such as warfarin (Coumadin), clopidogrel (Plavix), or aspirin, ask your doctor before taking omega-3 supplements.
- Bromelain, standardized, 40 mg three times daily. Although it's not a specific treatment for scleroderma, bromelain helps reduce pain and inflammation. It is often combined with turmeric. Bromelain can interact with a number of medications. It may increase the risk of bleeding, so if you take blood-thinners such as warfarin (Coumadin), clopidogrel (Plavix), or aspirin, ask your doctor before taking bromelain.
- Probiotic supplement (containing Lactobacillus acidophilus or bifidobacterium), 5 - 10 billion CFUs (colony forming units) a day. These "friendly" bacteria help maintain gastrointestinal health. One study found that probiotics helped reduce bloating in people with scleroderma that affected their digestive systems. Be sure to ask your doctor before taking probiotics, because some researchers think they could cause problems when you have an autoimmune disease.
Herbs may strengthen and tone the body's systems. As with any therapy, you should work with your health care provider before starting any treatment.
Very few studies have been done using these herbs to treat scleroderma. Ask your doctor before taking any of them.
- Turmeric (Curcuma longa), 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 works for scleroderma. It is often combined with bromelain. Turmeric may increase the risk of bleeding. If you take blood-thinners such as warfarin (Coumadin), clopidogrel (Plavix), or aspirin, ask your doctor before taking turmeric.
- Gotu kola (Centella asiatica), 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 really works. Gotu kola may interact with sedative medications, and may affect the liver. Ask your doctor before taking gotu kola.
A few studies suggest that acupuncture may improve blood flow in the hands and fingers, help heal fingertip ulcers, and maybe reduce the formation of fibrous tissue. It may also relieve pain.
Massage and Physical Therapy
Research suggests that massage may help improve circulation. More research is needed to know whether massage works for scleroderma.
Biofeedback may help some people with scleroderma better control the temperature in their hands and feet, although studies are mixed. Other mind-body techniques such as counseling, meditation, and emotional freedom technique (EFT) may help.
Prognosis and Complications
Possible complications include the following:
- Joint damage
- Damage to the digestive tract, leading to malnutrition
- Scarring of heart muscle; may lead to permanent damage
- Kidney damage and failure
The prognosis for people with scleroderma depends a lot on which form of the disease they have. For example:
- The CREST syndrome tends to worsen 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 get worse 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, the outlook is usually not good.
- Some people may have sudden remission from scleroderma, with symptoms going away in the reverse order that they appeared.
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