Scleroderma

Scleroderma

What is scleroderma?

Scleroderma, which literally means "hard skin", is a rare disorder of the body's connective tissues. It damages the cells that line the walls of the smaller arteries, such as those in the fingers and toes, and causes fibrous tissue to develop in the skin.

Scleroderma is usually distinguished in two forms - local scleroderma, which tends to affect the hands and face, and systemic scleroderma, which can damage the organs.

Localised scleroderma comes in two forms - morphea scleroderma, and linear scleroderma. Morphea scleroderma describes patches of hard skin forming on the skin and face. It develops slowly and can persist for a long time, but some people have known their morphea scleroderma to improve or even disappear.

Linear scleroderma causes stripes of hardened skin to develop across the face or arm. Occasionally, it can appear on the legs, and may affect muscle and bone tissue. In very rare cases, it can impair the growth of adolescents.

Systemic scleroderma is the more serious form of the illness. It is a progressive condition, which can affect the internal organs in addition to large areas of skin. Diffuse scleroderma is the most serious form of systemic scleroderma. It can cause damage to internal organs and is often diagnosed in conjunction with other autoimmune diseases, such as Lupus and Polymyositis.

Limited systemic scleroderma rarely affects the organs, but can be widespread and often leads to pulmonary tension. It is also known as CREST syndrome in America. CREST is an acronym that describes the group of related disorders characteristic of the illness.

 

  • Calcinosis describes the condition where small mineral deposits form under the skin around the joints. Skin ulcers can often form over the deposits.
  • Raynaud's disease precedes nearly all cases of scleroderma and is caused by abnormal changes in the small arteries and capillaries which result in constriction and a temporary interruption of circulation in the extremities, especially the fingers and toes.
  • Esophageal ( spelt oesophageal in Ireland ) motility dysfunction occurs when scleroderma scars the muscles in the oesophagus or gullet, impeding it from contracting normally.
  • Sclerodactylia is stiffness and tightening of the skin of the fingers. Bone damage may occur in the fingers and toes.
  • Telangiectasia is the dilation of small vessels and capillaries. This results in multiple thread like markings on the skin. They sometimes coalesce to form red markings particularly in the mouth and on the tongue.

How does scleroderma develop?

The causes of scleroderma are as yet unknown, although it is believed to be an autoimmune disorder. This means that the immune system becomes confused and begins to attack healthy cells in the body. A higher level of immune factors called anti-nuclear antibodies can be found in nearly all people with scleroderma. However, people with other autoimmune illnesses such as Lupus also show similar levels, as do around one in five of the healthy population.

How is scleroderma treated?

Because of the range of symptoms that scleroderma can manifest, and the varying rate with which it progresses, each person with scleroderma is likely to receive a different regime of treatment. The treatment is tailored for the particular symptom pattern of each patient. There are drugs available that can improve a number of the symptoms, and there is an operation, called a sympathectomy, which improves blood flow to fingers affected with Raynaud's phenomenon.

Your GP will advise you on the best method of treating an individual case of scleroderma. All patients can see some improvement in their symptoms by attempting to manage their condition.

How can I help manage my scleroderma?

Since most people with scleroderma initially experience the symptoms of Raynaud's disease, keeping the affected parts warm is essential. Fingers should be covered when there is a chance of touching cold surfaces, and you should wear a number of layers when going outside in cool weather.

Smoking is ill advisable for everyone, but people affected by scleroderma or Raynaud's are particularly at risk, since smoking aggravates the constriction that is already occurring from the condition. Quitting smoking and maintaining a smoke-free environment is essential for scleroderma patients.

Using moisturisers and antibiotic ointments can keep affected parts of the skin pliable and free from infection.

While there is no proof that vitamin supplements can help to treat scleroderma, some researchers in the United States have suggested that regular ingestion of vitamins A, C, and E can help as their anti-oxidant properties may counteract the effects of free radicals in the blood stream. These vitamins can more easily be obtained by eating portions of fresh fruit and vegetables daily.

Where can I get more information about scleroderma?

There is an organisation, which offers information and support for anyone suffering from Raynaud's disease or Scleroderma in Ireland:

Raynaud's & Scleroderma Ireland
Paradigm House, Dundrum Office Park, Dundrum, Dublin 14
Tel: (lo-call) 0818 363 999 | Fax: 01 2157945

Website - http://www.irishraynauds.com/

Email - info@irishraynauds.com


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