Scleroderma
About one person in 5000 has scleroderma, a generalised disease which produces fibrosis (thickening), degeneration and inflammation in the skin, blood vessels, muscles, and internal organs. The disease usually affects people 30 to 50 years old. Women are affected more often than men.
There are also localised forms of scleroderma, which involve only the skin.
Causes
Although the precise cause of scleroderma is unknown, the immune system is thought to be involved. Risk factors may include occupational exposure to silica dust and polyvinyl chloride (PVC). The course and severity of the disease varies widely in those affected.
Symptoms
Scleroderma may produce local or generalised symptoms. These can include:
- blanching, blueness or redness of fingers and toes in response to heat and cold (this is known as Raynaud's phenomenon);
- pain, stiffness, and swelling of fingers and joints;
- skin thickening, with shiny hands and forearms;
- tight mask-like facial skin;
- ulcers on fingertips or toes;
- heartburn;
- difficulty in swallowing;
- bloating after meals;
- weight loss;
- diarrhoea;
- constipation; and
- shortness of breath.
Diagnosis
Scleroderma is diagnosed by a combination of the patient's appearance, blood tests and sometimes through examining a biopsy (sample) of the skin. If the lungs are affected a chest X-ray might help.
Outlook
In most cases the disease progresses slowly. If the skin is the only part affected the outlook is better than if the digestive system, heart, lungs or kidneys are involved.
Last Reviewed: 07 March 2009
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