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What kind of Scleroderma

There are several kinds of scleroderma:
• Limited cutaneous scleroderma. Skin involvement is mainly limited to the forearm, hands and perhaps the face and neck. Internal organ involvement can occur, affecting the lungs and digestive system.

• Diffuse cutaneous scleroderma. Skin involvement is more extensive, affecting the arms all the way up to the shoulder. The skin over the chest, back and legs can also be affected. Internal organ involvement is usually more serious and extensive, potentially affecting the lungs, heart, digestive system, kidneys and blood vessels

• Morphoea. This is where a very limited area of skin is involved, usually with no internal organ involvement”

Scleroderma affects each individual differently. It is also possible that a small degree of overlap between the types of scleroderma can occur.

Who gets it?

Women are affected three to four times more often than men. The illness usually starts between the ages of 25 and 50. However, it sometimes affects children and the elderly.

What causes it?

The cause of scleroderma is unknown. It is not contagious, so you cannot catch it from someone or give it to anybody else. It is not inherited or passed on from one generation to the next. However, as is seen with many other diseases, some families seem more likely to get it than others.

Scleroderma is probably caused by a combination of different genetic and environmental factors. There is currently a lot of research on this.

How it starts and what takes place

One of the early symptoms is an extreme sensitivity to cold, and sometimes emotion, called Raynaud’s phenomenon. With Raynaud’s phenomenon, the blood vessels constrict or narrow, causing the fingers and toes to turn white and then blue. Often numbness and tingling is felt in the digits. This symptom is less common in hot climates like Malaysia. Most people with Raynaud’s do not get scleroderma, but most of those with scleroderma have Raynaud’s at some stage in their illness.

Another common early symptom of scleroderma is swelling or puffiness of the hands and feet, especially in the mornings. Your fingers and toes may look and feel swollen, the skin may appear shiny and the normal lines may disappear. The facial skin may seem stiff. About 95% of people with scleroderma have some hardening of the skin, especially of the hands, arms and face. Finger ulcers sometimes occur as a result of the narrowing of the small blood vessels. Some people may also develop small white calcium deposits in various parts of the body.

Scleroderma can also cause inflammation of the joints with symptoms of pain, stiffness, warmth and tenderness. Your may also get tired very quickly as the muscles become weak.

Contractures (when a joint tightens into a bent position) may occur due to tightening of the skin and involvement of the tissues surrounding the joints. This may take place in the fingers and elsewhere in the body.

The illness may also affect the tissues of the internal organs. For example, if the digestive system is involved, the muscles of the gullet may become weak causing problems in swallowing. Heartburn can be a frequent complaint. Sometimes, the middle and lower bowel is affected and bowel function is disturbed. The lungs may become inflamed which may result in scarring, leading to breathlessness. The heart and kidneys may also be involved.

Tests

There is no single test for scleroderma.

Treatment

There is no wonder drug or miracle cure for scleroderma. Treatment consists of exercise, care of the skin, and various medicines to help control the condition or treat complications.

An exercise programme is most crucial to reduce the effects of the illness. Regular exercise helps keep skin flexible, reduce contractures and keeps the blood flowing freely.

The only time that rest helps is if joints become inflamed. The physiotherapists and occupational therapists can help teach you an exercise programme and advice on the best ways of protecting your skin and joints.

Skin care is very important and the aim is to keep a good supply of blood flowing to the skin. Although Malaysia is a warm country, keeping yourself warm from head to toe helps open the blood vessels to the skin, especially in the hands and feet. You might find wearing socks in an air-conditioned room helpful. Do avoid using strong detergents or other substances that irritate your skin.

There are many brands of soap and creams designed to prevent dry skin; try some of these until you find one that gives you the best result.

Scleroderma affects people differently and to different degrees. Hence, what someone else is taking may not be right for you. Your doctor may prescribe treatment according to how the scleroderma has affected you.

There are emotional and social problems that come with having a chronic condition, and there may be times when you feel overwhelmed by problems. Emotional stress reduces blood flow and it is important to try to avoid undue tension. Do try and talk out any problems with your family and friends. If you need help in handling stress or depression, your doctor may be able to refer you to the right people who can help.

There is no evidence that diet either causes or has an effect on scleroderma. However, this condition can cause you to lose weight and some people have trouble with swallowing or with heartburn. Aim at having balanced meals and try to stay at your normal weight. If you have problems swallowing, eat slowly and chew thoroughly. Drinking water or some other liquid will soften food.

Eating several small meals rather than three large ones might make things easier. Raising the head of your bed helps if you suffer from heartburn. In this way, gravity will help keep acid from washing back from your stomach into the gullet while you sleep.

The type of medicine used to treat scleroderma depends on the type of organ involvement. Mild Raynaud’s phenomenon can be treated with a class of anti-hypertensive drug called calcium channel blockers. Severe Raynaud’s phenomenon can be treated with intravenous infusions of prostacyclin. Lung involvement can respond to steroids and immunosuppressant medicines. On the other hand, use of high dose steroids is associated with scleroderma renal involvement and should be avoided if possible.

The future

While scleroderma may change the way you look to some degree, it does not have to change the person that you are. Most people with this condition are still able to lead rewarding lives. Many things can be done to alleviate the condition. Most important is your own determination not to let it get the best of you, together with a common sense of approach.