Scleroderma

The name scleroderma literally means hard skin and this is the most obvious symptom of this long-term condition. There are two distinct forms of scleroderma:

  • localized scleroderma (sometimes called morphoea) which only affects parts of the skin and the tissues beneath it
  • systemic sclerosis which also affects other parts of the body including the joints, muscles, blood vessels, digestive system and occasionally the heart, lungs and kidneys.

When doctors refer to scleroderma it usually means the systemic type.

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Scleroderma symptoms

If you have scleroderma you produce too much collagen in the tissue connecting your organs. Collagen is an essential protein that helps hold your body together, but having too much can make the tissue of the body stiff and stop them working properly. This is most obvious in the skin because it can be seen on the outside of the body.

Scleroderma can cause a range of symptoms affecting many different parts of the body, including:

  • Raynaud's phenomenon - caused by narrowing of the blood vessels fingers or toes turn white, then blue in the cold. Just walking into a cold room or reaching into the fridge can make this happen. It is possible to have Raynaud's without having scleroderma, but most people with scleroderma will have symptoms of Raynaud's at some time during their illness, and it's often one of the first symptoms to appear
  • swelling of hands and feet, especially in the morning
  • shiny skin, without its usual creases
  • muscle weakness
  • thickening and hardening of the skin on the hands, arms and face – about 95% of people with scleroderma will notice these changes
  • thinning of the pads at the finger tips
  • open sores (ulcers) on the fingers, caused by poor blood supply
  • small, white chalky lumps under the skin, caused by calcium deposits
  • swollen, stiff, tender or painful joints. Some joints, such as your fingers, might tighten in a bent position (called a contracture)
  • digestive problems including difficulty swallowing caused by weakening of the muscles in the gullet (oesophagus), heartburn or bowel problems such as abdominal bloating, diarrhea or constipation.

Who gets scleroderma?

Scleroderma is a rare disease but women are 3–4 times more likely than men to get it.

Scleroderma usually starts between the ages of 25 and 55 and it only occasionally begins in children or in older people.

What causes scleroderma?

If you have scleroderma you produce too much collagen in the tissue connecting your organs. Collagen is an essential protein that helps hold your body together, but having too much can make the tissue of the body stiff and stop them working properly. This is most obvious in the skin because it can be seen on the outside of the body.

Small blood vessels will also be damaged by scleroderma. This could also be a result of excess collagen, which causes affected areas to thicken and stiffen, rather like scar tissue.

We don't yet know what causes some people to produce excess collagen, but it's thought to be a combination of genetic and environmental factors. The immune system – which normally fights off infections – appears to be overactive and attacks healthy body tissues instead.

Scleroderma isn't contagious, and is not passed on from one generation to another but some families do seem more likely to get it than others.

How does scleroderma progress?

Scleroderma is different for everyone, so it's hard to say how it might affect you. Most people find that scleroderma affects just a few parts of the body, starts slowly and gradually gets worse, but usually settles down to become stable after a few years. Sometimes it almost disappears after several years.

For some people the skin symptoms are the most troublesome, while others are affected more by poor circulation or digestive problems. And some people find their symptoms improve in summer but become worse in the winter.

Localized scleroderma doesn't develop into the systemic form of scleroderma.

Complications

A small number of people have more serious complications involving the lungs, heart and kidneys including:

  • scarring (fibrosis) of the lungs causing shortness of breath and/or a dry cough
  • narrowing of the blood vessels of the lungs leading to high blood pressure in the lungs (pulmonary hypertension) which may in turn strain the heart
  • kidney problems resulting in high blood pressure.

However, these complications are relatively rare and can all be treated. Regular check-ups are recommended so that any problems can be identified at an early stage.

How is scleroderma diagnosed?

There is no single test for scleroderma, and the characteristic thickening of the skin is often the key factor in making the diagnosis. However, tests can be helpful in establishing whether other parts of the body are involved.

Tests could include:

  • blood tests
  • x-rays
  • breathing tests
  • a heart scan (echocardiogram)
  • a skin biopsy, where a small piece of skin is removed and examined under a microscope.

After you've been diagnosed, you might need to see your doctor regularly, and have more tests every now and again to monitor the disease and to check for early signs of the more serious complications.

Scleroderma treatment

There is no drug that will cure scleroderma. But a combination of medications and self-help treatments can help to control the symptoms and treat any complications.

Scleroderma drugs

There are a number of drugs that can be used to help with the various symptoms:

  • Drugs such as nifedipine can help with symptoms of Raynaud's phenomenon by widening the blood vessels, thus improving blood circulation to the hands and feet
  • Creams applied to the skin, or an infusion called iloprost given into a vein through a drip, can help with painful ulcers
  • Antacids can be useful for digestive problems such as heartburn and swallowing difficulties
  • Non-steroidal anti-inflammatory drugs (NSAIDs) should help to relieve joint pain and inflammation
  • Immunosuppressive drugs may be used in more severe cases of scleroderma, especially if the lungs are involved or for more extensive skin involvement
  • Drugs such as ACE inhibitors can be used to treat high blood pressure
  • Steroid tablets may be used, usually in low doses, either in the early stages of the disease where the skin is just starting to look puffy, or later on if the muscles or lungs are affected.

Self-help and daily living

People with scleroderma can make their daily lives easier by eating healthily, looking after the skin and by exercising regularly.

This section also covers: aids and gadgets, looking after you skin and dealing with stress.

Exercise

One of the best things you can do to keep on top of scleroderma is to follow a regular exercise programme. This will keep your skin flexible, reduce any tightening in your joints, and keep your blood flowing freely.

Your physiotherapist will be able to teach you the best exercises to protect your skin and joints.

The only time rest will help is if your joints become swollen. In that case, temporary lightweight splints might help protect them and reduce contractures.

Diet and nutrition

Diet isn't thought to cause scleroderma or to have much effect on the condition. But you may lose weight because of the disease, or have trouble with swallowing or heartburn.

  • Eat six small meals a day instead of three larger ones – this helps with digestion while ensuring that the body receives the nutrients it needs
  • Eat slowly, chew thoroughly and drink plenty of water with meals
  • Taking your largest meal in the middle of the day can help to avoid heartburn
  • Don't eat too much in the evening to ensure time for digestion
  • Raise the head of your bed a few inches to stop acid coming back up from your stomach into your oesophagus while you sleep.

Supports, aids and gadgets

If you struggle to open childproof medicine containers, ask your pharmacist to put your drugs in containers you can manage. Contact us for a special request card you can hand over with your prescription.

Looking after your skin

You need a good supply of blood flowing to your skin to stop it from cracking, peeling and developing ulcers.

  • Keep warm from top to toe – this will help open the blood vessels to your arms, hands, legs and feet. Wear a hat to help preserve your body heat. Thermal clothes, hand warmers and electrically-heated gloves and socks can also help
  • If your skin is broken or painful, dressings can help to protect it
  • Don't use strong detergents or anything else that irritates your skin. Try soaps, creams and bath oils designed to prevent dry skin until you find the ones that give you the best results in keeping your skin supple
  • Smoking reduces the blood flow to your skin so it's best to stop.

Dealing with stress

Stress can reduce the blood flow to some parts of your body, so it can affect your disease, particularly if you have Raynaud's phenomenon.

Talking about any feelings of stress or depression with your family and friends can help, or your doctor might be able to treat you, or refer you to a social worker or counsellor.

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