The symptoms of scleroderma generally differ depending on the type of scleroderma: localized or generalized.

Localized scleroderma
If you have localized scleroderma, usually only the skin on your hands and face will be affected. The course of localized scleroderma is very slow and it rarely becomes more widespread or causes severe complications. Localized scleroderma takes on two forms:

Morphea (pronounced mor-fee-ah) scleroderma is the form where hard round or oval patches develop on the skin. These patches tend to be white with a reddish area around them. They may occur on your chest, stomach, face, arms, legs or other parts of your body.

With the linear form of localized scleroderma, a line of thickened skin may occur in areas such as your face (often the forehead), arms or legs. This line of hard skin extends deep down into the skin, bones and muscles, and may alter growth of the affected part. This form usually occurs in young people who develop scleroderma.

Generalized scleroderma
With generalized scleroderma (sometimes called systemic scleroderma) the organs of your body, widespread areas of your skin, or both may be involved. Generalized scleroderma also occurs in two different forms: limited (also called CREST syndrome) and diffuse scleroderma. Both of these are slowly progressive, meaning they get worse over time.

With the form of generalized scleroderma called limited scleroderma, your skin hardening may be widespread, but not everywhere on your body, and it will not appear on your trunk. Often with this form the lungs, heart and kidneys are not involved. Limited scleroderma is also known as CREST syndrome. CREST stands for a number of different conditions that commonly occur with limited scleroderma: calcinosis (pronounced cal-si-no-sis), Raynaud's phenomenon (pronounced ray-noze feh-naw-meh-non), esophageal (pronounced i-sof-ah-jee-ul) dysfunction, sclerodactyly (pronounced scler-o-dac-tul-lee), and telangiectasia (pronounced tell-an-jee-ek-stas-ee-ah). One or more of these conditions can also occur in other forms of scleroderma.

Calcinosis is the name given to the condition where small white chalky lumps made up of calcium form under the skin. These lumps often form around the fingers but can occur anywhere in the body. They can rupture through the skin and cause a chalky white material to ooze out. Eating too much calcium does not cause these lumps.

Raynaud's phenomenon is caused by a spasm or narrowing of the blood vessels in your limbs. Exposure to the cold or emotional stress can trigger such spasms. The resulting reduction in blood flow causes your fingers and toes to become blue or white in colour. If the condition is severe you may also have pain and numbness in the affected areas. Smoking aggravates this condition. Raynaud's phenomenon occurs with many other medical problems apart from scleroderma.

Esophygeal dysfunction results when scleroderma affects the esophagus (pronounced i-sof-a-gus).  Food is carried from your mouth down your esophagus to your stomach.  In your esophagus a rhythmic muscle action moves the food along. With scleroderma your esophagus can become less mobile and the muscle action can diminish. Over time, the muscle may be replaced by scar tissue. This can result in difficulty swallowing. You might also get heartburn more often as the acid from the stomach is able to flood upwards into the esophagus where it can cause burning. Some people also experience a feeling of bloating when eating and a desire to vomit after eating.

The word sclerodactyly means 'hardness of the digits.' With this condition the skin of your fingers may become dry, coarse-textured or feel 'woody.' Hair may vanish from your hands, the creases on them may disappear, and they may look shiny. You may also have difficulty moving your fingers and they may tighten into a bent position.

Telangiectasia is a condition where small red spots appear on the surface of the skin.  These are due to enlarged (damaged) blood vessels. They commonly occur on the fingers, palms, face, lips and tongue.

A sixth, but less common symptom is also associated with CREST. You could develop bowel dysfunction, which leads to an inability to absorb nutrients from food, an overgrowth of bacteria in your bowel, weight loss and diarrhea or constipation. Bowel dysfunction occurs when the muscles of your intestine weaken and are replaced by scar tissue, as can happen with your esophagus.

If you have diffuse scleroderma , the other form of generalized scleroderma, you will probably experience widespread thickening of the skin on your arms, legs, face, chest and stomach. These skin changes can occur quite rapidly, though progress of this form of the disease varies widely from person to person. In addition to the skin changes, other areas are frequently involved: lungs, kidneys, heart, blood vessels, bowel, esophagus, joints, and connective tissues such as tendons and ligaments. Diffuse scleroderma can become very severe -- even life threatening. It may overlap with other autoimmune diseases, including lupus (pronounced loo-pus) and polymyositis (pronounced pol-ee-my-o-site-iss). If this occurs the disorder is then referred to as mixed connective tissue disease.

Heart and lung problems may result from diffuse scleroderma. Usually it is the left pumping chamber of the heart that is involved. Problems with your heart could include a slowing of your heart rate or changes in its rhythmic pattern. Heart failure can also occur. If your lungs are involved, they will lose their ability to function efficiently. Symptoms of heart or lung involvement may include shortness of breath, coughing, chest pain and irregular beating of the heart.

If your kidneys are affected by scleroderma blood flow to them may be restricted. This can produce high blood pressure and kidney failure and is a serious problem requiring urgent treatment. Symptoms could include severe headache, shortness of breath, vision problems, chest pain and mental confusion.

The glands and organs that produce fluid can also be affected. Dry eyes, mouth or vagina may indicate that you have a condition called Sjögren's (pronounced show-grens) syndrome, which may accompany scleroderma. Your eyes may become dry if the glands that produce tears, the lacrimal glands, fail. The salivary glands in your mouth can also fail. In a woman with diffuse scleroderma the cells that normally keep her vagina moist may fail and the area can become abnormally dry.

The joints are not usually the primary area of involvement in scleroderma, but inflammation of the joints can occur as part of the disease. The skin, when involved with thickening and tightness, also lead to stiffness and reduced motion of the joints in the affected areas.

Diagnosis may be difficult as there is no specific test that confirms scleroderma is present.  Your doctor will probably perform a physical examination and order other tests such as x-rays and blood tests. 

If skin changes are already present the diagnosis may be simple and routine. In other cases where scleroderma is suspected, your doctor may wish to do a skin biopsy, which involves removing a small piece of skin to be analyzed in a laboratory. Your doctor may feel your skin, checking for thickened and hardened areas, and may also press tendons and joints to detect crackling or grating sensations, which can indicate changes beneath the skin. A major clue to diagnosing scleroderma is the symmetrical (happening in the same spot on both sides of the body) hardening and thickening of the skin in areas on the fingers and toes. By looking at your fingernails underneath a microscope, your doctor may also be able to detect changes in your blood vessels that are characteristic of scleroderma and mixed connective tissue disease.

There are a wide variety of other tests possible, which may be done based on the organs that are involved. For example, if you're having difficulty swallowing your doctor may order a special X-ray of the esophagus.

If you are diagnosed as having scleroderma, the goal of your treatment plan will be to bring the symptoms and disease under control. Treatment plans are based on the type and severity of symptoms, and are individualized to meet each person's needs. Your active involvement in developing your prescribed treatment plan is essential.

Medicine

NSAIDs reduce pain when taken at a low dose, and relieve inflammation when taken at a higher dose. NSAIDs such as ASA (Aspirin, Anacin, etc.) and ibuprofen (Motrin IB, Advil, etc.) can be purchased without a prescription. Examples of NSAIDs that require a prescription include Naprosyn, Relafen, Indocid, Voltaren, Feldene, and Clinoril. The various NSAIDs and Aspirin®, if taken in full doses, usually have the same levels of anti-inflammatory effect. However, different individuals may experience greater relief from one medication than another. Taking more than one NSAID at a time increases the possibility of side effects, particularly stomach problems such as heartburn, ulcers and bleeding. People taking these medications should consider taking something to protect the stomach, such as misoprostol (Cytotec).

Cortisone is a steroid that reduces inflammation and swelling and that can influence regulation of the immune system. It is a hormone naturally produced by the body. Corticosteroids are man-made drugs that closely resemble cortisone. 

The most common form of corticosteroid is called prednisone, taken in pill form. Prednisone use needs to be carefully monitored because of its many side effects, and the drug must never be stopped abruptly. Some of the side effects from long-term use include cataracts, high blood pressure, sleep problems, muscle loss, bruising, thinning of the bones (osteoporosis), weight gain and increased risk of infections. The goal with this and most drugs is to find the lowest effective dose that will avoid as many of the side effects as possible.

DMARDs target the processes causing the inflammation, but do not reverse permanent damage. The most common of them are gold salts, methotrexate, sulfasalazine, hydroxychloroquinine, chloroquinine and azathioprine. Methotrexate is often used to treat scleroderma. DMARDs are usually given in addition to other medications such as NSAIDs. They usually take a few months to make a difference in the inflammation. Side effects may include mouth sores, diarrhea and nausea. More serious side effects, monitored through regular blood and urine tests, include liver damage, and excessive lowering of the white blood cell count (increasing susceptibility to certain infections) and platelet count (affecting blood clotting).

Penicillamine is a medication that is also sometimes used in the treatment of rheumatoid arthritis. Researchers have recently been investigating its possibility of treating skin thickening and preventing abnormal cell growth in the lungs.

A variety of other agents may be used depending upon the particular organs involved. Medications that control small blood vessel spasms are used in individuals with marked Raynaud's phenomenon. In cases where kidney involvement has lead to high blood pressure, certain medications need to be used to deal with this potentially serious problem. Medications are available to reduce acid production in the stomach and control heartburn. Other drugs may improve bowel problems.

Body and skin care

If you have scleroderma you should do things to care for your skin. Using a humidifier in your house during winter months will help prevent the air, and your skin from becoming too dry. Topical creams can be used to prevent your skin from drying out. Light mineral oil, petroleum jelly, and cocoa butter are moisturizers that work well. It may also be necessary for you to limit the frequency of bathing to preserve your natural skin oils.

Try to protect areas of skin calcification from pressure and abrasion. In these hardened areas the skin can more easily break down and get infected. Keep areas of skin that may have opened very clean. Aggressive treatment of infections, should they occur, is important.

Another goal of skin protection is to maintain good circulation. By wearing gloves and warm socks in cold weather, your skin is protected from cold air, which reduces the risk of the blood vessels shrinking (vasospasm). If reaching into the freezer for food triggers Raynaud's phenomenon try wearing gloves or oven mitts during this activity. If you have the symptoms of Raynaud's phenomenon and smoke, stop smoking. Smoking triggers vasospasms in your whole body and will worsen the symptoms.

By carefully and adequately chewing your food you can minimize your difficulty swallowing. Drinking fluids with food will assist if the mouth is dry. There are some medications that can assist in stimulating the muscle action of the esophagus. If your esophagus has narrowed, it is possible to have it periodically stretched.

Raising the head of your bed, and not lying down right after meals can help you minimize acid coming back up into your esophagus. Eating smaller meals more frequently will also help.
 
If you have the symptoms of Sjögren's Syndrome, there are a variety of lubricants you can use to make up for the lack of secretions. For example, artificial tears can be used as drops in your eyes. There are also mouthwashes and sprays available to increase the moisture in your mouth.

Where dryness of the mouth is pronounced, it is recommended that you have regular dental checks as there may be less flushing of the teeth than is usual. This can lead to increased tooth decay.

Exercise

• Regular exercise will help you to keep the blood flowing in areas that are affected by scleroderma. 
• Muscles and the other tissues that hold joints together weaken when they aren't moved enough, so the joint loses its shape and function. Doing certain exercises can help keep your muscles strong and joints moving.

There are different types of exercises:

  Always consult your doctor before beginning an exercise program.

Protect your body and joints

Protecting your joints means using them in ways that avoid excess stress. Benefits include less pain and greater ease in doing tasks. Three main techniques to protect your joints are:

Pacing, by alternating heavy or repeated tasks with easier tasks or breaks, reduces the stress on painful joints and allows weakened muscles to rest.

Positioning joints wisely helps you use them in ways that avoid extra stress. Use larger, stronger joints to carry loads. For example, use a shoulder bag instead of a hand-held one.  Also, avoid keeping the same position for a long period of time. 

Using helpful devices, such as canes, luggage carts, grocery carts and reaching aids, can help make daily tasks easier. Small appliances such as microwaves, food processors and bread makers can be useful in the kitchen. Using grab bars and shower seats in the bathroom can help you to conserve energy and avoid falls.

Relaxation

Developing good relaxation and coping skills can give you a greater feeling of control over your arthritis and a more positive outlook.

Surgery

Where lack of blood circulation is a marked problem with scleroderma, surgical sympathectomy is sometimes done. During this procedure the nerves in the spine that causes spasms in the blood vessels are divided to lessen the chance of future spasms.

The Cochrane Musculoskeletal Review Group (CMSG) is a specialized group of researchers and consumer representatives that belong to the Cochrane Collaboration, an international not-for-profit organization that encourages informed decisions about health care by preparing, maintaining and promoting reviews of the effects of various health care treatments.

CMSG members review the best available literature to determine the best evidence to support specific arthritis treatments. They explore the evidence for and against the effectiveness and appropriateness of treatments (medications, surgery, education, etc) in specific circumstances. The results are medical reviews that are then included in The Cochrane Library and made available to health professionals around the world.

For members of the public who also want to be informed about the evidence concerning health care practices and decisions in their lives, the Cochrane Musculosketal Review Group have summarized their reviews into consumer fact sheets. For the Cochrane consumer reviews related to scleroderma, visit www.arthritis.ca/cochrane.

Along with the physical symptoms of arthritis, many people experience feelings of helplessness and depression. Learning daily living strategies to manage your arthritis gives you a greater feeling of control and a more positive outlook. To get the best results, people affected by arthritis need to form close ties with their doctors and therapists, and become full partners in their treatment. From our perspective, it's all part of 'living well with arthritis.' There are several resources you can use in finding out how best to manage your own arthritis. Here are a few:

• The Arthritis Self-Management Program (ASMP) is a unique self-help program offered by The Arthritis Society to help you better control and manage your arthritis.
• The Open Forum within this Web site is an opportunity to discuss and share information with other visitors - people who, through their own experiences, may be able to offer some useful insights.

Of course, there are many other valuable resources for people with arthritis. If you're unclear about where to look for help, be sure to call The Arthritis Society at 1-800-321-1433.