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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.
The millions of cells that make up the human body are held together by meshes of connective tissue. This mesh is made up of special substances – collagen, elastin and proteoglycans. Collagen fibres are extremely strong, elastin fibres are ‘stretchy’ and proteoglycans cannot be compressed. An overproduction of collagen occurs in scleroderma, causing hardening and tightening of this connective tissue.
Many different areas of the body can be affected but, generally, the connective tissue of the skin and internal organs (such as the heart, lungs and kidneys) become hard and tight.
Anyone can develop scleroderma, but it is more common between the ages of 20 and 40, and three times more likely in females. Estimates vary, but it is possible that as many as one in 5,000 people may have some degree of 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:
- Limited scleroderma - this indicates that hardening of the skin occurs only below the elbow and on the face. Other symptoms include Raynaud’s phenomenon, stiffness of muscles or joints and oesophageal reflux (a type of heartburn). Usually, these symptoms can be well managed.
- Diffuse scleroderma - this indicates that hardening of the skin is more widespread and internal organs are more severely affected. Symptoms can include Raynaud’s phenomenon, oesophageal reflux, hardening of the skin (of the hands, forearms, torso, upper arms and thighs), diarrhoea or constipation and lung or kidney impairment. This is a more serious form of the disorder. Diffuse scleroderma can also occur without skin hardening.
Other categories of scleroderma that are sometimes referred to include:
- Localised – this refers to scleroderma that occurs only in the skin and underlying muscles and bones in discrete areas. There is no organ involvement and no Raynaud’s phenomenon.
- CREST – this stands for the symptoms of Calcinosis (calcium deposits in the skin), Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly (hardening and tightening of the skin of the fingers) and Telangiectasis (small red marks commonly on the skin of face and hands). It does not involve any skin hardening, although it can occur in combination with the other forms of scleroderma.
- Overlap – this refers to cases in which scleroderma occurs with another autoimmune disease such systemic lupus erythematosus or rheumatoid arthritis.
The immune system may play a role
Special cells called fibroblasts make the protein collagen, which gives connective tissue its strength. In a person with scleroderma, the fibroblasts make too much collagen, which builds up and thickens the connective tissue. The exact reason is not known, but it is suspected that the immune system may be affecting the fibroblasts.
A person with scleroderma has autoantibodies circulating in their blood. Antibodies are made by the immune system to kill foreign invaders like bacteria. An autoantibody is an antibody designed to attack a part of the body itself. Other immune system cells, such as lymphocytes, are overproduced in people with scleroderma.
The reasons why the immune system is affected, its role in the disease and the reasons behind the overproduction of collagen are not known. There does not appear to be a genetic element to the disease.
Common complications
A person with scleroderma can develop a number of complications, including:
- Joint pain, swelling and stiffness
- Osteoporosis, caused by immobilised joints
- Osteolysis, the shortening of the fingers caused by a reabsorption of bone
- Muscle wastage
- A build-up of collagen in the muscle tissues
- Thickening of the tendons
- Symptoms similar to carpal tunnel syndrome
- Sjogren’s syndrome – an autoimmune condition that damages sweat and saliva glands and causes dry eyes and mouth
- Digestive problems – the muscles in the oesophagus (the tube which leads from your mouth to your stomach) may become weak. This can cause difficulty with swallowing. Heartburn is a common problem and bowel function may be disturbed
- Complications such as kidney failure or hardening of the lung tissue (in severe cases, where internal organs are affected).
Diagnosis and treatment
Scleroderma is diagnosed using a range of medical tests including physical examinations, blood tests including a test for autoantibodies (antinuclear antibodies or ANA) and tissue biopsies.
There is no cure for scleroderma. Treatment aims to ease the symptoms and will be dependent on the individual’s symptoms. Different medications can be prescribed for joint and muscle pain, Raynaud’s phenomenon, kidney impairment, lung involvement and heartburn.
A person with scleroderma should be monitored by a rheumatologist or immunologist. Other health care providers, such as dermatologists and gastroenterologists, may take part in ongoing treatment.
Managing 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 the person cope with the demands and challenges of their disease.
- Aids and equipment – the effects of scleroderma may make some actions difficult: for example, opening door handles or getting dressed. Changing the home or work environment and using aids and equipment can help. An occupational therapist can provide advice.
Where to get help
- Your doctor
- The Scleroderma Foundation of Victoria Tel. (03) 9288 3651
- Arthritis Victoria inc. Osteoporosis Victoria Tel. (03) 8531 8000 or 1800 011 041
- Rheumatologist
- Immunologist
- Dermatologist
Things to remember
- Scleroderma is an incurable disease of the connective tissue.
- The disorder can be mild, moderate or severe, depending on how much skin and connective tissue is affected.
- Treatment is limited to slowing the progression of the disease and easing the symptoms.
You might also be interested in:
Immune system.
Marfan syndrome.
Polymyositis.
Raynaud's phenomenon.
Sjogren's syndrome.
Skin explained.
Want to know more?
Go to More information for support groups, related links and references.
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