Scleroderma – an Auto-Immune Disease

Scleroderma comes from the Greek words sclero which means hard, and derma, relating to the skin. Although it was first described in 1753, it was only in 1847 that it was recognised as a condition that affected the skin, and gained the name of scleroderma.

What is Scleroderma?

This condition is a chronic, multi-system auto-immune disease, in which the body over-reacts to its own tissues, rather than fighting off foreign substances such as bacteria or viruses. In the case of scleroderma, not only the skin, but also the gut, lungs and kidneys can be affected.

There are two main types of scleroderma, which is also known as progressive systemic sclerosis. In localised sclerodrema only the skin is affected, whilst in the systemic variation, the internal organs such as the gut and lungs are involved. It is rare for someone with localised scleroderma to later develop systemic disease.

What Does Scleroderma Do?

The disease causes the body to increase its production of collagen, which is a normal component of the skin, ligaments and tendons. Too much collagen results in thickening and tightness. The following symptoms can also be present.

Raynaud’s syndrome, which can lead to finger ulcers.
Reduced oesophageal mobility and reflux.
Telangiectasias – small red spots, often on the face, caused by widened blood vessels.
Arthritis of the fingers with oedema or swelling of the joints with loss of movement.
Tightening and loss of elasticity of the skin leading to a ‘pinched’ look to the face.
Pulmonary fibrosis.
Kidney disease.
Bowel disease.

Who Will Develop Scleroderma?

There are about 6-7000 people with scleroderma in the UK, and some 300,000 people affected in the USA. The most frequent age of onset is 25 to 55, and women are more likely to develop the disease than men. It is also more common in native American and African American’s than it is in Caucasians. There does appear to be a genetic link, but it is not clear what that link is yet. There is a connection with other auto-immune diseases.

What is The Treatment for Scleroderma?

There is no cure for scleroderma, and treatment is geared towards preventing complications. Maintaining the elasticity of the skin is important, and physiotherapy can help to prevent joints becoming too stiff. Non-steroidal anti-inflammatories may be useful for arthritic hands. Laser therapy may help with the telangietasias, but they can return.

In general, prevention of problems is important, with protection of the hands and feet during cold weather, as well as stress reduction, joint protection and pacing to avoid fatgiue.