Scleroderma

Thickening and hardening of the connective tissues in the skin, joints, and internal organs

  • Most common between the ages of 30 and 50
  • More common in females
  • Sometimes runs in families
  • Lifestyle is not a significant factor

Scleroderma, also known as systemic sclerosis, is a rare disorder in which the connective tissues that hold together the structures of the body become in-flamed, damaged, and thickened. The tissues, particularly those in the skin, then contract and harden.

This condition is an autoimmune disorder in which the body produces antibodies that attack and damage its own connective tissues. The reason for this reaction is unknown, but genetic factors may play a part since the condition may sometimes run in families. Scleroderma is approximately four times more common in women than it is in men and occurs most commonly in adults under the age of 50.

What are the symptoms?

Scleroderma commonly affects the skin and the joints, but other organs may be involved. Symptoms vary from mild to severe and may include:

  • Fingers or toes that are sensitive to the cold, becoming white and painful (see Raynaud’s phenomenon and Raynaud’s disease).

  • Ulcers and small, hardened areas that appear on the fingers.

  • Swollen fingers or hands.

  • Pain in the joints, especially the joints in the hands.

  • Thickening and tightening of the skin, which is most severe on the limbs but may affect the trunk and face.

  • Muscle weakness.

  • Difficulty swallowing due to stiffening of the tissues of the oesophagus.

If the lungs are affected, shortness of breath may develop. In some people, scleroderma causes high blood pressure (see Hypertension) and eventually kidney failure.

Effects of scleroderma

In a person with scleroderma, the skin on the fingers and hands may become thickened and swollen, making it difficult to straighten the fingers.

What might be done?

Your doctor may be able to make a diagnosis from your symptoms and from a physical examination. He or she may also arrange for blood tests to look for certain antibodies. A small skin sample may also be taken for examination.

There is no cure for scleroderma, but treatment can slow the progression of the condition, reducing the damage to body organs and relieving symptoms. If the lungs are affected, an immunosuppressant drug may be prescribed. Calcium channel blockers may be prescribed to treat Raynaud’s phenomenon, and your doctor may also advise you to wear gloves and socks to protect your fingers and toes from the cold. If your muscles or joints are affected, a corticosteroid may be prescribed.

The course of scleroderma is variable. Scleroderma that mainly affects the skin tends to be milder, with the internal organs being less severely affected. However, when several organs are affected, the condition tends to be more severe and can be life-threatening.

From the 2010 revision of the Complete Home Medical Guide © Dorling Kindersley Limited.

The subjects, conditions and treatments covered in this encyclopaedia are for information only and may not be covered by your insurance product should you make a claim.

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