Scleroderma (which comes from the Greek meaning ‘hard skin’) is a connective tissue disease that affects the skin and other major internal organs.
It is an autoimmune condition, involving the overproduction of collagen and blood vessel damage. Excess collagen is laid down in these organs which in turn results in scarring and reduced normal function of the affected organs. Scleroderma can cause physical disability and be life threatening as the skin, joints, tendons, and parts of internal organs can all be affected. Autoimmune conditions occur when the body's tissues are attacked by its own immune system.
- Around 1 in 10,000 people in the UK have scleroderma, with women affected four times as often as men. There are approximately 8,000 people with scleroderma in the UK. The complex nature of the condition can often lead to misdiagnosis, so the true number of people affected may be much higher.
- The onset of scleroderma is most frequent between the ages of 25 to 55 but it may affect any age group, from infants to the elderly.
- The exact cause or causes is still unknown. Factors other than gender, such as race and ethnic background, may influence the risk of developing scleroderma, as well as the age of onset, and the pattern or severity of internal organ involvement.
- There are 2 main groups of scleroderma, and within are further subsets:
1. Systemic sclerosis - the systemic form of scleroderma, which affects the internal organs as well as the skin.
2. Localised scleroderma - a type of scleroderma that affects specific areas of skin and underlying tissues, but does not involve the internal organs.
- Symptoms can vary widely from person to person, but usually include hardening of the skin, swelling of the hands and feet, joint pain and stiffness and blood vessel damage leading to a physical over-reaction to cold or stress. The heart, oesophagus, blood vessels, kidneys, lungs, blood pressure and digestive system can all be affected resulting in a variety of other symptoms, some of which can be life-threatening or cause physical disability.
- According to their severity, the symptoms of scleroderma can be treated with drug therapy, physical therapy, surgery and self-management techniques.
There is currently no cure but proper treatment and management can make it possible for many people with scleroderma to lead full and productive lives.
Comprehensive information on all aspects of scleroderma is available in our booklet:
Understanding and Managing Scleroderma
More detailed information on different problems in scleroderma can be found in our information leaflets.