The Scleroderma Association of NSW Inc. cannot offer direct advice on treatment or available therapies. It is up to each patient to make all relevant health decisionsIn consultation with their own doctor or health professional.|
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What is Scleroderma?
It is an acquired, rare, non-contagious disease that occurs world wide in sporadic cases, with a great variety of clinical presentations.
Scleroderma literally means "Hard Skin".
It is a disease that affects skin particularly, but also internal organs. It may be difficult to understand because it is not one disease. It may be a family of ten or twenty different diseases. There are three major categories of Scleroderma; Localised, Limited/Crest, and Diffuse. Limited and Diffuse are both forms of Systemic Scleroderma - a fact that is often misunderstood.
Symptoms:
Thinning of the lips and furrowing of the mouth
Loss of motility in the oesophagus resulting in a difficulty in swallowing
Lungs can be affected resulting in a difficulty
in breathing
Heart may be affected
Stomach affected causing heartburn
Kidneys affected causing renal failure
Bowels affected causing diarrhoea
Tight skin, thickened and shiny skin
Prominent red blood vessels
Loss of skin marks and hair
Who can get Scleroderma?
Incidence of Scleroderma
Scleroderma can occur at any age. It is 4 times more common in women than men, except during childbearing years when it is 10 times more common in women than men. It tends to affect people most commonly between the ages of 25 to 55. Scleroderma can occur occasionally in children and the elderly. In children it is more likely to be the linear form of scleroderma.
Scleroderma appears to affect people of any race and ethnic background, and in any part of the world. It is an uncommon disorder, and affects approximately 1-2 people in every 10,000. Scleroderma is not directly inherited but there is evidence to indicate that in very rare circumstances some families may have certain genetic tendencies towards the disease.
Scleroderma literally means "Hard Skin". It is a chronic degenerative disorder that leads to the overproduction of collagen in the body's connective tissue.
In More Details
SCLERODERMA (Systemic Sclerosis)
Scleroderma is a disorder of the connective tissue of the body. The word Scleroderma comes from two Greek words: "Sclero" meaning hard, and "derma" meaning skin. It was originally thought to be a skin disorder, because of the hardening of the skin, which mainly affects the hands and feet. However, it is now realised that the blood vessels and some internal organs can also be involved. Scleroderma is an uncommon disorder and affects:
*
The skin, usually the hands and feet, may become thickened, tight, tough and shiny due to swelling and thickening of the connective tissue.* The thickening is found in most people with scleroderma, and may sometimes spread to other areas, such as the arms, face, trunk and legs.
The blood vessels may be narrower than normal, and react to changes in temperature by going into spasm when the fingers or toes are exposed to the cold.
There are different forms of Scleroderma - localised and systemic.
Localised Scleroderma has two types: Morphoea and Linear
Morphoea affects the skin, and forms thickened patches or plaques which are usually oval in shape. They can get bigger or shrink and sometimes disappear altogether. They are more common on the trunk but can occur on the face, arms or legs. This type of scleroderma may last a few months or persist for years.
Linear scleroderma usually develops in childhood, and can affect the growth of a limb. It starts as a band-like thickening of the skin, and usually limited to a particular area, such as an arm, a leg, or forehead. It differs from morphoea, in that it can involve deeper layers of the skin, as well as the surface layers, and sometimes the mobility of underlying joints may be affected.
Systemic Sclerosis
The term systemic sclerosis is used to describe two forms of scleroderma:
* Limited Systemic Sclerosis * Diffuse Systemic Sclerosis
They differ from one another in a variety of ways. It is now possible to distinguish between the two types, by the amount of skin and internal organ involvement, and by particular markers detected in the blood. Both scleroderma or systemic sclerosis can be used. They have the same meaning.
Limited Systemic Sclerosis
About 60% of all people with scleroderma have this type of scleroderma. It was previously known as "CREST" syndrome as it represented the initials of 5 common symptoms of this type of scleroderma.
Diffuse Scleroderma
Skin changes in people with this type of scleroderma, tends to be more widespread than the limited form. It often involves the skin of the trunk, as well as upper arms and lower legs. Internal organs are more likely to be involved also, such as the lungs, heart, muscles, joints and the kidneys.
General Symptoms
There may be general symptoms associated with scleroderma, such as tiredness, lack of energy, general weakness, aching muscles, joints and bones.
Features of Scleroderma
The symptoms of scleroderma tend to vary considerably from person to person. Often the first symptom is Raynaud's phenomenon. This can occur many years before the onset of other symptoms.
Raynaud's Phenomenon
Raynauds Phenomenon
may well be the first symptom anyone has. It may be months or years before other systems develop. Because scleroderma is rare and often hard to diagnose, it is important to screen people who have Raynaud's. This way those people who may be at risk can be given treatment, or be closely monitored by their doctor.Patients undergo a general change in health and well-being eg: joint stiffness and aches, muscle weakness, loss of weight, tiredness - even in the morning, shortness of breath, reflux, problems with blood circulation in fingers and toes associated with numbness and sensitivity to cold temperatures (Raynaud's), high blood pressure with kidney disease.
The hands and feet of people who have Raynaud's phenomenon are extremely sensitive to the cold. The blood vessels respond to the cold, or sometimes emotional stress, by narrowing down, allowing very little blood to supply the finger tips. The fingers feel numb, extremely cold, and are white in colour. The narrowing lasts only a short time, and the fingers may then become blue and finally quite red as the supply of blood returns to normal. It's common for the fingers to tingle whilst they are returning to normal and they may be painful.
It is important to understand that Raynaud's phenomenon can more commonly occur on its own and is not an indication of any underlying condition. Many people, especially women, experience Raynaud's phenomenon and have no evidence of a connective tissue disorder.
Other areas may also be affected, such as the ear lobes, nose and tip of the tongue. Raynaud's phenomenon probably affects the kidneys, lungs and heart in a similar way.
The Skin, Muscles and Joints
The thickening of the skin over the fingers is called sclerodactyly. It may be mild and almost unnoticed. In other people, the thickening makes their skin feel tight, dry and puffy. The thickening, due to an increase in collagen, may make it difficult to move the hands properly, so that people are unable to make a full fist. Ulcers may also occur on the finger tips, causing pain, especially if they become infected. These ulcers are often very slow or difficult to heal due to the poor circulation. Very dry skin may also lead to ulceration.
Small red spots called telangiectasia may appear on the skin and inside the mouth, They occur mainly on the hands and face. They are completely painless and are caused by a collection of tiny blood vessels matted together. Once they have formed, they do not get bigger or bleed. They can be camouflaged by make up. Occasionally small white lumps of calcium can be deposited just under the skin, They are relatively uncommon, and not everyone with scleroderma will be affected in this way. These lumps are known as calcinosis. They can sometimes break through the skin, causing pain, particularly if they become infected. Calcinosis is most likely to occur on the fingers. Sometimes the skin around the mouth can become tight, making lip and mouth movements difficult. Mouth stretching exercises can be very helpful in maintaining facial and mouth movements.
Digestive Problems
People with scleroderma often experience problems with digestion, particularly in the upper part of the digestive tract.
When food is swallowed it passes down the gullet or oesophagus and into the stomach. The action of muscles in the oesophagus pushes the food downwards into the stomach. These muscles may become weakened and the oesophagus itself may narrow, making the passage of food through it much more difficult. There is a special valve, (called a sphincter) where the oesophagus enters the stomach. This valve normally opens to allow food to enter the stomach and then closes again, preventing regurgitation. Scleroderma can affect this valve so that it fails to close completely. This allows food to pass back into the oesophagus, taking acid from the stomach with it. The acid causes a burning sensation when this occurs. Long-term this can lead to damage and scarring of the lower end of the oesophagus.
The muscles of the small bowel may be affected by scleroderma causing them to be less efficient in pushing food through the digestive tract. This allows the small amount of bacteria normally present, to overgrow. This can lead to feelings of bloating and pain. As the muscles are not working well, less food is absorbed leading to weight loss and possible anaemia. Bacterial overgrowth may also lead to diarrhoea.
If scleroderma affects the large bowel, then constipation may result as the weakened muscles cannot push the contents along normally. This can cause many problems for people with scleroderma. In a small number of people, it may lead to faecal incontinence, as there may be involvement of the sphincter around the anus. Treatments for this problem are available, including medications, pelvic floor exercises, and occasionally surgery.
The Lungs
The lungs may be affected in some people with scleroderma. Symptoms include some shortness of breath, particularly after exercise, and there may be a persistent dry cough. There are 2 ways that scleroderma affects the lungs. One is due to the thickening of the lung tissue itself, preventing the lungs from expanding and contracting as easily as they would normally do. This is called pulmonary fibrosis. Sometimes the blood vessels to the lungs may be affected, causing them to be narrower than usual. This can lead to high blood pressure to the lungs which decreases their capacity to work properly. This high blood pressure to the lungs is called pulmonary hypertension.
The Heart
The heart is affected in a very small minority of people with scleroderma. It may interfere with the electrical impulses of the heart or sometimes, the heart muscle itself.
The Kidneys
The kidneys may be affected in people with scleroderma. It is more likely to occur in people who have a diffuse systemic type of scleroderma. Kidney involvement is often indicated by a rise in blood pressure. In order to avoid kidney damage it is important to treat high blood pressure quickly. There are now medications available that can control blood pressure extremely well. Monitoring blood pressure is a quick and easy way of checking that blood pressure is under control.
Sjogren's Syndrome see our Sjogren's Syndrome pamphets
This causes dryness in the mouth and eyes due to decreased production of saliva in the mouth, and tear production in the eyes. The dryness in the eye may cause bouts of conjunctivitis, and a feeling of grit in the eyes. Increased dryness in the mouth may lead to swallowing difficulties, and an increase in tooth decay. Dry eyes respond well to artificial tears, and a dry mouth is often helped by a good intake of fluid and keeping the mouth moist by chewing things like sugarless gum. Vaginal dryness may occur as a result of Sjogren's syndrome, making intercourse painful. A simple lubricant available from chemists can help to relieve this symptom.
Causes of Scleroderma
The cause of scleroderma is not known though there are several factors such as genetics, the immune system, and environmental triggers that may play a role in the condition. The initial changes that occur involve the overproduction of collagen in the skin, tendons, blood vessels and some internal organs. This over production of collagen causes thickening and hardening of the affected areas. It is possible that some alterations in the body's defence system or immune system may play a part in the onset of the condition. It is also known that people who carry certain genetic markers tend to be more susceptible to developing scleroderma.
Environmental Causes
Some environmental factors are now known to be possible important triggers for the development of a scleroderma like condition. Some workers in the PVC (Plastics) industry using polyvinyl chlorides have developed a scleroderma like condition. Substances such as silica, and epoxy resins may also cause a condition which has the features of scleroderma. Contaminated rapeseed oil, sold as olive oil in Spain in the early 1980's resulted in many people dying of a condition called Toxic Oil Syndrome. Some of the survivors of this condition went on to develop a scleroderma type illness. Scleroderma caused by these environmental factors appears to respond better to treatment than scleroderma from an unknown cause.
It is important to recognise that for most people with scleroderma, the cause is unknown, and that environmental factors may not be involved at all.
Diagnosis of Scleroderma
The diagnosis of scleroderma is usually made by a physical examination, and the person's medical history. Diagnosis is not always straightforward mainly because some of the symptoms of scleroderma may also be present in other conditions. A good example is Raynaud's phenomenon which is also found in people with systemic lupus erythematosis (Lupus), rheumatoid arthritis, polymyositis (sore muscles) and other connective tissue diseases.
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The Scleroderma Association of NSW Inc.
cannot offer direct advice on treatment or available therapies.
It is up to each patient to make all relevant health decisions
In consultation with their own doctor or health professional.
Updated Updated Friday, 18. April 2008
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