The word ‘scleroderma’ means ‘hard skin’. Scleroderma affects the connective tissues of the body (tissues that hold together joints, muscles, blood vessels and internal organs). The connective tissues of people with scleroderma have too much of a protein called collagen. Collagen is important to give connective tissue its strength, but excess collagen causes hardening and tightening of the affected area. Many different areas of the body can be affected. There are two major types of scleroderma: "limited diffuse scleroderma" (CREST Syndrome) and scleroderma or systemic sclerosis.
Scleroderma is an autoimmune condition. The normal role of your body’s immune system is to fight off infections and diseases to keep you healthy. In an autoimmune disease like scleroderma, your immune system starts attacking your own healthy tissues. This stimulates the production of excess collagen.
What are the symptoms?
Symptoms vary greatly from person to person and also depend on what part of the body is involved. Symptoms may include any of the following:
- Thickening and hardening of the skin, particularly on the fingers, arms and face.
- Raynaud’s phenomenon: 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, in response to cold weather. 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.
- Small white chalky lumps (calcium deposits) under the skin.
- Stiffness and pain in the muscles and/or joints.
- Indigestion or heartburn.
- Diarrhoea or constipation.
- Shortness of breath or reduced ability to exercise.
What causes it?
The exact cause of scleroderma is unknown. Genetics, the immune system and environmental factors may play roles in causing this condition. This has yet to be proven in research.
How is it diagnosed?
There is no specific test for scleroderma. Your doctor will diagnose scleroderma from your symptoms, a physical examination and various tests, such as blood tests or a skin biopsy (a small piece of skin is removed and examined under a microscope). It may take several visits before your doctor can tell if you have scleroderma as the symptoms can overlap with other diseases and types of arthritis.
What treatments are there for scleroderma?
Many of the symptoms of scleroderma can be improved with medicines and/or lifestyle changes. Your doctor will tailor your treatment to your symptoms and how severe your condition is. 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.