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Scleroderma


Signs and Symptoms
Causes
Risk Factors
Diagnosis
 
Preventive Care
Treatment
Other Considerations
Supporting Research

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." Scleroderma occurs 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:

Localized scleroderma

  • 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.

Systemic scleroderma

  • 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

Causes

Doctors believe scleroderma is caused by the immune system mistakenly attacking the body's own tissues. The immune system attack causes 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. Both genetics and environment may play a role.

Risk Factors

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.

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 do a physical examination and feel your skin, checking for thickened and hardened areas. The doctor may also press affected tendons and joints. The doctor may also do the following procedures:

  • 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.

Preventive Care

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

Treatment

There is no cure for scleroderma. Medication can treat symptoms and may help prevent complications. Lifestyle and dietary changes can 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 affect circulation.
  • Use soothing skin creams to reduce pain, swelling, and stiffness.

Medications

Localized scleroderma often is treated with moisturizers applied to the skin or corticosteroids. Oral medications such as minocycline (Minocin or Dynacin) may also be used to stop 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 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
    • 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, 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 gastrointestinal system. Ask your team of health care providers about the best ways toad complementary and alternative therapies into your overall treatment plan. 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 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, and processed foods.
  • Avoid caffeine, 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. Omega-3 supplements may increase your risk of bleeding. If you take blood-thinning medications such as warfarin (Coumadin) or aspirin, ask your doctor before taking omega-3 supplements.
  • Bromelain, standardized, 40 mg three times daily. Although not a specific treatment for scleroderma, bromelain helps reduce pain and inflammation. It is often combined with turmeric. Bromelain may increase the risk of bleeding. If you take blood-thinning medication such as warfarin (Coumadin) or aspirin, ask your doctor before taking bromelain.

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. It is often combined with bromelain. Turmeric may increase the risk of bleeding. If you take blood-thinning medication such as warfarin (Coumadin) or aspirin, ask your doctor before taking turmeric.
  • 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 really has any benefit. Gotu kola may interact with sedative medications, and may affect the liver. Ask your doctor before taking gotu kola.

Acupuncture

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

Massage and Physical Therapy

Research suggests that massage may help improve circulation. More research is needed to know whether massage is really an effective therapy for scleroderma.

Mind-Body Medicine

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.

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 a lot on which form of the disease they have. 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 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, prognosis is generally poor.
  • Some people may have sudden remission from scleroderma, with symptoms going away in the reverse order that they appeared.

Supporting Research

Amento EP. Immunologic abnormalities in scleroderma. Semin Cutan Med Surg. 1998;17(1):18-21.

Arlett CM et al Identification of fetal DNA and cells in skin lesions from women with systemic sclerosis. N Engl J Med. 1998;338(17):1186-91.

Baur JA, Sinclair DA. Therapeutic potential of resveratrol: the in vivo evidence. Nat Rev Drug Discov. 2006;5(6):493-506.

Black CM. Scleroderma in children. Adv Exp Med Biol. 1999;455:35-48.

Burckdorfer KR, Hillary JB, Bunce T, et al. Increased susceptibility to oxidation of low-density lipoproteins isolated from patients with systemic sclerosis. Arthritis Rheum. 1995;38(8):1060-1067.

Casale R et al Systemic sclerosis (scleroderma): an integrated challenge in rehabilitation. Arch Phys Med Rehab. 1997;78(7):767-73.

Cunningham BB, Landells, IDR, Langman C, et al. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol. 1998;39(2 Pt 1):211-215.

Elst EF, van Suijlekom-Smit LWA, Oranje AP. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children. Pediatr Dermatol. 1999;16(1):53-58.

Fauci AS, Braunwald E, Hauser SL, et al, eds. Harrison's Principles of Internal Medicine. 17th ed. New York, NY: McGraw-Hill; 2008.

Gelber AC, Wigley FM. Treatment of scleroderma. Curr Opin Rheumatol. 1995;7(6):551-9.

Hale LP, Greer PK, Trinh CT, James CL. Proteinase activity and stability of natural bromelain preparations. Int Immunopharmacol. 2005;5(4):783-93.

Haustein UF, Anderegg U. Pathophysiology of scleroderma: an update. J Eur Acad Dermatol Venereol. 1998;11(1):1-8.

Herrick AL, Worthington H, Rieley F, et al. Dietary intake of micronutrient antioxidants in relation to blood levels in patients with systemic sclerosis. J Rheumatol. 1996;23(4):650-653.

Herrick AL. Advances in palliative care for the patient with scleroderma. Curr Opin Rheumatol. 1996;8(6):555-60.

Herron GS, Romero LI. Vascular abnormalities in scleroderma. Semin Cutan Med Surg. 1998;17(1):12-7.

Hunnicutt SE, Grady J, McNearney TA. Complementary and alternative medicine use was associated with higher perceived physical and mental functioning in early systemic sclerosis. Explore (NY). 2008 Jul-Aug;4(4):259-63.

Hunzelmann N et al. Management of localized scleroderma. Semin Cutan Med Surg. 1998;17(1):34-40.

Jimenez SA et al. Pathogenesis of scleroderma. Rheum Dis Clin North Am. 1996;22(4):647-74.

Kartnig T. Clinical applications of Centella asiatica (L.) Urb. Herbs Spices Med Plants. 1988;3:145–173.

Kerin K, Yost JH. Advances in the diagnosis and management of scleroderma-related vascular complications. Compr Ther. 1998;24(11-12):574-81.

Maeda M, Kachi H, Ichihashi N et al. The effect of electrical acupuncture-stimulation therapy using thermography and plasma endothelin (ET-1) levels in patients with progressive system sclerosis (PSS). J Dermatol Sci. 1998;17(2):151-155.

Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 1996;22(4):751-64.

Mitchell H, et al. Scleroderma and related conditions. Med Clin North Am. 1997;81(1):129-49.

Olsen NJ, et al. Muscle abnormalities in scleroderma. Rheum Dis Clin North Am. 1996;22(4):783-96.

Pang BK, Munro V, Kossard S. Pseudoscleroderma secondary to phytomenadione (vitamin K1) injections: Texier's disease. Australas J Dermatol. 1996;37(1):44-47.

Rose S, et al. Gastrointestinal manifestations of scleroderma. Gastroenterol Clin North Am. 1998;27(3):563-94.

Silver RM. Scleroderma: clinical problems: the lungs. Rheum Dis Clin North Am. 1996;22(4):825-40.

Simopoulos AP. Omega-3 fatty acids in inflammation and autoimmune diseases. J Am Coll Nutr. 2002;21(6):495-505.

Sjogren RW. Gastrointestinal features of scleroderma. Curr Opin Rheumatol. 1996;8(6):569-75.

Van den Hoogen FH, de Jong EM. Clinical aspects of systemic and localized scleroderma. Curr Opin Rheumatol. 1995;7(6):546-50.

Wang HK. The therapeutic potential of flavonoids. Expert Opin Investig Drugs. 2000;9(9):2103-19.

White B. Immunologic aspects of scleroderma. Curr Opin Rheumatol. 1995;7(6):541-5.

Wollina U, Abdel-Naser MB, Mani R. A review of the microcirculation in skin in patients with chronic venous insufficiency: the problem and the evidence available for therapeutic options. Int J Low Extrem Wounds. 2006;5(3):169-80.

Yoon JH, Baek SJ. Molecular targets of dietary polyphenols with anti-inflammatory properties. Yonsei Med J. 2005;46(5):585-96.


Review Date: 9/2/2010
Reviewed By: Steven D. Ehrlich, NMD, Solutions Acupuncture, a private practice specializing in complementary and alternative medicine, Phoenix, AZ. Review provided by VeriMed Healthcare Network.
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