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What is Scleroderma?
Scleroderma International Foundation (SIF)

What is Scleroderma?

Scleroderma is a chronic disease of unknown origin involving the circulatory system, connective tissue (particularly the skin), and the immune system. One of the most visible manifestations is the build-up of scar tissue in the skin, and the term scleroderma means "scarred or hardened skin." Current thinking and research about the underlying disease process in scleroderma focuses on three areas of involvement: thecirculatory system, connective (scar) tissue,and the immune system.

The Circulatory Hypothesis

Raynaud's Phenomenon -- frequent spasms of the small arteries induced by cold and emotion -- is characteristic of scleroderma. Although most apparent in the color changes -- white, red and blue -- in the fingers and occasionally the toes, the spasms are now known to be occurring in various areas throughout the body, including the internal organs. These spasms can deprive tissues of normal circulation for extended periods. The resulting damage is thought to stimulate repair processes -- primarily the formation of scar tissue (sclerosis) that results in hardening of the tissues in the affected areas.

The original cause of the Raynaud's remains unknown. It could be a virus, an allergy, a hormonal disorder; very few hypotheses have been completely ruled out.

Connective Tissue Abnormalities

Research suggests that scleroderma involves abnormal behavior of the body's connective tissues. The make-up of the scar tissue build-up in scleroderma is different than is found after more routine injury repair processes. However, since the scleroderma damage-process is somewhat unique, it is still unclear whether the abnormalities of scar tissues are cause or effect.


There is considerable evidence of autoimmunity as part of the disease process in scleroderma as is the case in other Rheumatic Diseases such as Lupus and Rheumatoid Arthritis. In autoimmunity -- immunity directed against the self -- the immune system attacks (rejects) normal tissue. In scleroderma, autoimmunity may occur because there is so much damaged tissue in the body that the immune system is overwhelmed and becomes confused as to which tissue proteins are "self" and which are foreign. Other causes may include a virus. In any case, therapies that suppress the immune system have shown clear symptomatic relief.

What causes Scleroderma?

The fundamental cause of scleroderma remains unknown. We do know that it is not contagious, so you can't catch it from someone or give it to anyone. Except in rare instances it is not inherited or passed on from one generation to the next. There may be an important link between scleroderma and exposure to toxic substances, such as silica dust or polyvinyl chloride, but research in this area has been minimal.

Although stress certainly appears to play an important role in the timing of the onset of scleroderma, it is unclear whether it should be considered causative. More research needs to be done in this area. After the onset, patients are chronically sensitive to stressful situations.

Although some progress has been made in treating various symptoms, no effective treatment or cure for the overall disease has been discovered.

Who gets Scleroderma?

The current estimate is that there are 500,000 to 700,000 people in the United States with scleroderma, with about three to four thousand new patients per year. A similar percentage of the populations in other countries have scleroderma.

Women are two or three times more likely to fall victim to scleroderma than are men. The disease usually starts in the prime of life, between ages 25 and 55. Because of the female/male ratio and this most common period of onset, there is suspicion of a hormonal factor, although preliminary research has been unable to develop a specific hypothesis. Scleroderma develops occasionally in children and the elderly.



Are there different types of Scleroderma?

There are two commonly recognized forms of scleroderma:

Localized scleroderma (Linear and Morphea)

A disorder of the connective tissue limited to the skin, the tissues just beneath the skin, and muscle. It does not affect the internal organs. Localized scleroderma is relatively mild, and may be related to systemic scleroderma only in terms of similar superficial symptoms (appearance of skin biopsy under the microscope).

Systemic Scleroderma (sclerosis)

Involves the skin as well as many internal body parts, including blood vessels, digestive system (esophagus, stomach and bowel), lungs, kidneys, muscles and joints. There have been attempts to define subcategories in systemic scleroderma that might help us predict the course of the disease in individuals.

The so-called CREST pattern is one example. CREST Syndrome is a relatively benign and chronic variation of scleroderma that is usually not serious and almost never fatal. The disease is characterized by calcium deposits, usually in the fingers; Raynaud's Phenomenon (previously described); loss of muscular control of the esophagus which can cause difficulty in swallowing; sclerodactylia, a tapering deformity of the bones of the fingers; and teleangiectasia, small red spots on the skin of the fingers, face, or inside of the mouth. Although some physicians still believe this is a useful sub-categorization, the existing research studies have been unable to predict consistently how (or whether) the disease will progress in any specific individual.

Another subcategory of systemic scleroderma is Sjogren's Syndrome. Sjogren's Syndrome causes inflammation of the conjunctiva (the mucous membrane covering the anterior surface of the eyeball and the lining of the lids) and the cornea, dryness of the mucous membranes, dilation of vessels or spots (red, then purple) on the face, and/or enlargement of the parotid (a gland behind the ear). Sjogren's Syndrome also causes changes in the tear and salivary glands.

Linear scleroderma can appear on the arms, legs, or forehead as a long streak resembling a deep sabre wound -- often called "en coupe de saber." This occurs most often in children.

Morphea is characterized by hardened, slightly depressed areas of thickened skin. The areas are whitish or yellowish white in color, surrounded by a pinkish or purplish halo. The condition is referred to as "generalized morphea" when the plaque-like lesions are widespread on the skin. Underlying structures, especially in the extremities, often contract and atrophy with generalized morphea.

What are the symptoms of systemic Scleroderma?

Systemic scleroderma can affect any part of the body, and take very different courses in different people. Consequently, there are a great variety of symptom patterns. Because of this, diagnosis -- particularly in the early stages -- has been, and remains, a major problem.It is common for scleroderma patients to experience a series of piecemeal diagnoses and treatments before their physician detects the overall pattern of symptoms suggesting scleroderma. The first step in diagnosis should be a thorough physical examination and history of past and present symptoms.One characteristic early symptom is Raynaud's Phenomenon (previously described). However, although over ninety percent of scleroderma victims have this constriction of small blood vessels, only about one in fifty people with Raynaud's ever develops scleroderma. One recent study indicated that perhaps as many as twenty percent of all women report experiencing Raynaud's during some period of their lives.About 95 percent of people with scleroderma show some early thickening and hardening of the skin, especially of the hands, arms and face. This is due to the increased production of fibrous scar tissue. Other early symptoms include:

Swelling of hands and feet
Numbness of hands and feet
Shiny skin and disappearance of skin folds
Ulcers on the fingers
Calcium deposits on the fingers
Joint inflammation
Joints tightening into bent position
Muscle weakness
Itchy skin
Difficulty in swallowing
Shortness of breath
Fatty diarrhea or constipation
Loss of body hair
Many scleroderma patients experience CREST Syndrome and Sjogren's Syndrome (previously described)

Microstomia is another common problem associated with scleroderma. Microstomia causes the skin around the face and mouth area to become hard and tight. Mobility of the the tongue is reduced as well. Occasionally scleroderma may affect only the connective tissue of the digestive system or some other internal body system, leaving the skin and joints untouched. With digestive system involvement, swallowing doesn't work normally. Heartburn is often a problem and sometimes the bowel is affected.

The heart, lungs, and kidneys may also become involved in scleroderma. One of the earliest symptoms reported by scleroderma patients is heart irregularities. Formation of scar tissue in the heart and lungs has recently been recognized as a general pattern probably contributing to overall fatigue. Kidney involvement is the most serious and life threatening complication. As a result, high blood pressure, which may occur, is extremely dangerous but treatable with medications.










How is Scleroderma treated?

There is no known cure for the overall disease process in scleroderma. There are new drugs which have demonstrated long term effects. However, to a limited extent it is possible to treat the more common symptoms. There are new drugs to reduce the frequency and severity of Raynaud's Phenomenon. Several other drugs are used to treat skin problems, and still others are beneficial in treating high blood pressure. Always check with your doctor to find out which treatment is best for you.

Impressive long-term survival (longevity) benefits were recently reported for scleroderma patients treated over a period of years with PABA (para-amino benzoic acid). Both animal and clinical research using anti-oxidant therapies such as vitamin E and Dimethyl Sulfoxide (DMSO) and Dimethyl Sulfone (MSM) have both directly and indirectly demonstrated long-term benefits.

Dimethyl sulfoxide is specifically approved for scleroderma in both Canada and Russia. Anti-oxidants (or free radical scavengers), ex hypothesis, may serve to partially protect tissues from damage and assist less sclerotic repair processes.

Immune-suppressive agents such as D-penicillamine have shown good initial results, while others such as cyclosporine (known for its use in suppressing rejection of organ transplants) represent the leading edge of research trials. D-penicillamine is also a significant anti-oxidant.

Antacids may be recommended for chronic heartburn. Aspirin is commonly prescribed to help relieve pain and reduce inflammation. Other therapies produce benefits for other specific symptoms.

Side Effects and Sensitivities

Scleroderma patients, on the average, seem to be more sensitive to the side effects of most medications than the average person. Extra precations are appropriate even in normal drug therapy procedures.

Health Promotion

Exercise is very important in keeping scleroderma under control. Physical therapy sessions are very helpful. Regular movement and exercise helps keep skin flexible and promotes better blood flow.

Nutritionally balanced meals are especially important because intestinal absorption may become impaired and because stress depletes the body's store of vitamins. Avoiding certain foods, such as chocolate, may be helpful in alleviating heartburn. Doctors recommend that patients with scleroderma refrain from smoking. Smoking causes the blood vessels to constrict and reduce blood flow, adding to the overall problem.

Emotional stress tends to have the effect of further reducing blood flow. Therefore, scleroderma patients need plenty of sleep and should try to avoid stressful situations. Many people with scleroderma find that counseling is useful. Meditation, demonstrated to help in other stress related circulatory diseases, may be worth considering.

Group support from a local scleroderma organization can have a really dramatic effect. The chance to talk about problems and frustrations can be just what is needed to improve spirits and help find solutions.

What haven't I heard of Scleroderma before?

Scleroderma has only recently gained really significant medical attention. Perhaps this is because it develops slowly and the onset is not usually dramatic, or because life-threatening complications were formerly thought to be uncommon.

Scleroderma is also difficult to diagnose. Many cases in the past were not fully recognized and physicians tended to treat isolated symptoms only.

Finally, it may be because scleroderma is relatively rare. However, it victimizes more people than muscular dystrophy, a disease most people have heard of through the Jerry Lewis Telethon. The good news is that scleroderma has recently attracted major medical interest both among basic researchers and clinicians.

How can I help?

The Scleroderma International Foundation (SIF) needs help to:

  1. Reach out to people isolated with scleroderma
  2. Develop education/support groups
  3. Raise funds to promote new research efforts
  4. Increase public awareness of scleroderma
  5. Strengthen physician training to further prompt diagnosis and improve therapy
  6. Support The Connector, the newsletter of the Scleroderma International Foundation, which is issued quarterly with updated material concerning research and treatment.

The efforts to eradicate major diseases like scleroderma have always been financed by patients, their families and a concerned public. To achieve our goals, we need your support.

You can help to conquer scleroderma. Fund-raising is very important to the Scleroderma International Foundation. And your generous donation is, of course, tax deductible. If you are interested in helping, please get in touch with us at the below contact information.


Dr. Stanley Jacob

Contact Dr. Stanley Jacob in his office in Portland, OR at (503) 494-8474,or email Dr. Jeffrey Tyler at jmueller@dmso.org.

DMSO Organization wishes to thank the Scleroderma International Foundation (SIF) for allowing this article to be place on our World Wide Web site. SIF retains all copyright. Please contact the Scleroderma International Foundation (SIF) for reprint information or permission.