Scleroderma

Also known as systemic sclerosis

The name scleroderma literally means 'hard skin'. Scleroderma is a rare, chronic, often progressive autoimmune disease which affects the connective tissues of the body (muscles, joints, blood vessels and internal organs).

Key points

  1. Scleroderma affects 4x as many women as men.
  2. Average age at diagnosis is around the 40's.
  3. There is no cure for scleroderma, but many treatments are available for specific symptoms.
  4. Lifestyle measures are also helpful.
  5. It is not hereditary, so rarely occurs in more than one family member.

The connective tissues of people with scleroderma have too much of a protein called ‘collagen’. Collagen is important to give connective tissues its strength, but excess collagen causes hardening and tightening of the affected area.

Types of scleroderma

There are two major types of scleroderma:

  • Localised scleroderma (sometimes called ‘morphea’), which affects the skin and sometimes the tissues beneath it (for example, muscle). More commonly starts in childhood.
  • Systemic scleroderma, which also affects the other parts of the body, including blood vessels, joints, the digestive system (oesophagus, stomach and bowel), and occasionally the lungs, heart and kidneys. Systemic scleroderma is more common in adults. The systemic form has two subtypes:
    • Limited scleroderma, including CREST syndrome, where the skin involvement is mainly limited to the hands and there is a lower chance of internal organ involvement.
    • Diffuse scleroderma, in which skin involvement is generalised and organs are often involved.

Causes

The cause of scleroderma is not known. We do not yet know why people’s immune system becomes overactive and starts making too much collagen, but it is thought to be a combination of genetic and environmental factors.

Symptoms

Symptoms vary greatly from person to person depending on what part of the body is involved. Symptoms may include any of the following:

  • Raynaud’s phenomenon where the fingers or toes turn white, then blue in the cold, and then red as blood flow returns. This is caused by narrowing of the blood vessels. It is possible to have Raynaud’s without having scleroderma, but most people with scleroderma will have symptoms of Raynaud’s at some time and it is often one of the first symptoms to appear.
  • Thickening and hardening of the skin on the hands, arms and face.
  • Stiffness and pain in the muscles and/or joints.
  • Swelling of the hands and feet, especially in the morning.
  • Thinning of the pads at the finger tips.
  • Small, white, chalky lumps (calcium deposits) under the skin.
  • Indigestion or heartburn.
  • Diarrhoea or constipation.
  • Shortness of breath or reduced ability to exercise.
  • Kidney problems and high blood pressure.

How does scleroderma progress?

Scleroderma is different for everyone. For most people scleroderma starts slowly, affecting just a few parts of the body, gradually getting worse, but usually becomes stable after a few years.

  • Some people find their symptoms improve in summer but become worse in winter.
  • People with more severe forms of scleroderma can have serious problems with organs such as the kidneys, lungs and heart.
  • If these organs are affected, you may need to see a specialist (for example, a kidney specialist if your kidneys are affected).
  • Localised scleroderma does not develop into the systemic form.

Diagnosis

There is no single test for scleroderma, an experienced doctor bases diagnosis primarily on a person’s medical history and a physical examination. 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:

Treatment

At present there is no cure for scleroderma, but many treatments are available for specific symptoms. There is no way of predicting exactly which treatment will work best for you. Your doctor may need to trial several different treatments and medicines before finding the one that is right for you.

Self care

Ways you can help yourself:

  • Learn about scleroderma: scleroderma differs from person to person but can be treated effectively, discuss your treatment options with your doctor and healthcare team.
  • Exercise: one of the best things you can do is to follow a regular exercise regime. It will help to keep your joints flexible and improve blood flow. Your physiotherapist will design a programme to protect your skin and joints.
  • Manage Raynaud’s phenomenon: avoid, where possible, exposure to cold and sudden temperature changes. Keep your whole body warm and protect your hands and feet with gloves and warm socks.
  • Look after your skin: avoid strong detergents that can irritate your skin, keep your skin clean and well-lubricated to prevent dryness and infection.
  • Avoid cigarette smoke: smoking reduces the blood flow to the skin.
  • Manage stress: ensure you get sufficient rest and relaxation, and balance work and leisure; for support talk to your health professionals, family and support groups.
  • Healthy diet: including frequent small meals rather than the usual large meals, these may help if you have trouble with swallowing or heartburn.

Learn more

Scleroderma Arthritis NZ
Treatment and symptomatic management Patient Info, UK
Scleroderma page NHS Choices
What is scleroderma? Scleroderma Foundation US

Credits: Editorial team. Last reviewed: 19 Jan 2015