Scleroderma

About scleroderma

Scleroderma is a disease of the body's connective tissue. Scleroderma means 'hard skin'. The most common symptom is a thickening and hardening of the skin, particularly of the hands and face.

Connective tissue holds together and supports our joints, muscles, internal organs and skin. The connective tissue of people with scleroderma has too much collagen, causing it to harden and tighten.

Many different areas of the body can be affected by scleroderma, and symptoms vary greatly from person to person.

Anyone can develop scleroderma, but it is more common between the ages of 30 and 60, and three times more likely in females. There are estimated to be over 5,000 Australians living with scleroderma.

Scleroderma is not contagious. The condition can be mild, moderate or severe. There is no cure, but it is possible to successfully manage the symptoms.

Types of scleroderma

There are two broad classifications of scleroderma based on the amount of skin and other organs affected. These are:

• localised scleroderma – affects the skin. There are two types of localised scleroderma, morphea or linear. Both have distinctive signs and symptoms. Localised scleroderma can sometimes restrict normal joint movement, due to hardening of the skin over the joint. The cause of localised scleroderma is unknown
• diffuse scleroderma – (also called systemic scleroderma or systemic sclerosis) involves a more widespread hardening of the skin. Internal organs are also affected. Systemic sclerosis is an autoimmune disease, meaning that the condition arises due to immune system overactivity, whereby the immune system attacks the body’s own tissues.

Symptoms of scleroderma

The symptoms will vary from person to person and will depend on the type of scleroderma and which internal organs are involved, if any.

Symptoms can include:

• thickening and hardening of the skin
• Raynaud's phenomenon – a condition which affects the blood flow to the extremities, most often fingers and toes. It is caused by a sudden constriction of the blood vessels
• stiffness and pain in the muscles or joints (or both)
• indigestion, heartburn, diarrhoea, constipation.

Causes of scleroderma

The cause of scleroderma is not known. Possible causes may include an overactive immune system, genetics and environmental triggers, although none of these factors has been proven.

Diagnosis of scleroderma

There is no specific test for scleroderma, so your doctor will use a range of medical tests including physical examinations, blood tests and tissue biopsies to reach a diagnosis.

Treatment of scleroderma

There is no cure for scleroderma. Treatment aims to ease the symptoms and will be dependent on the person's symptoms. Steroid creams applied to the skin may be prescribed for localised scleroderma. A range of other anti-inflammatory medications may also be prescribed. Specialised medications are also available for scleroderma-associated lung disease.

A person with scleroderma should be monitored by a dermatologist or rheumatologist. Other healthcare providers such as immunologists, respiratory physicians and gastroenterologists may take part in ongoing treatment.

Management of scleroderma

Managing the disease depends on its severity, but may include:

• medication – to decrease the symptoms of Raynaud's phenomenon or treat other affected organs such as the gastrointestinal tract, lungs or kidneys
• lifestyle changes – avoid exposure to cold temperatures, dress warmly and don't smoke. These measures help manage Raynaud's phenomenon
• gentle, regular exercise – to counteract the effects of joint immobilisation and improve overall health
• stress management – to help you cope with the demands and challenges of the disease
• aids and equipment – the effects of scleroderma may make some actions difficult, for example, using door handles or getting dressed. Changing your home or work environment and using aids and equipment can help. An occupational therapist can provide advice.

Where to get help

• Your GP (doctor)
• Scleroderma Victoria
• Musculoskeletal Australia Tel. (03) 8531 8000 or 1800 263 265