What is Scleroderma?

What causes Scleroderma?

How is Scleroderma treated?

How can I help manage my 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 generally classified into two forms;

- local scleroderma - which tends to affect the hands and face, and

- systemic scleroderma - which can damage the organs

Systemic scleroderma is a progressive condition and therefore a more serious form of the condition, affecting the internal organs in addition to large areas of skin.

Diffuse scleroderma is a further sub-type of systemic scleroderma where excess collagen production leads to skin thickening over large areas of the body. It can also cause serious 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 - 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. Blood supply to the extremities, fingers and toes usually, becomes interrupted as the arteries that normally supply them spasm and contract.
• Esophageal (spelt oesophageal in Ireland) - motility dysfunction occurs when scleroderma scars the muscles in the oesophagus or gullet, impeding it from contracting normally.
• Sclerodactylia - stiffness and tightening of the skin of the fingers and toes, bone damage may occur.
• Telangiectasia - dilation of small vessels and capillaries which results in multiple thread like markings on the skin. They sometimes join together to form red markings, particularly in the mouth and on the tongue.

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What causes Scleroderma?

The causes of scleroderma are unknown, although it is believed to be an autoimmune disorder. Which means the body's natural self-defence system, gets confused and starts to attack your body's healthy tissues. 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.

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How is scleroderma treated?

Each person living with scleroderma is likely to have a different treatment plan. This is due to the range of symptoms scleroderma can present with and the varying speed with which it progresses. The treatment is individualised to the symptoms exhibited by each person.

There are drugs available that can improve a number of the symptoms, and your GP will advise 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.

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How can I help manage my scleroderma?

As nearly all people who develop scleroderma will initially experience the symptoms of Raynaud's disease, keeping the affected parts warm is essential. When going outside in cool weather, always wear a number of layers and cover your fingers if you are likely to come into contact with cold surfaces.

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

The use of moisturising creams/gels and antibiotic ointments is recommended, as this can keep affected parts of the skin pliable and infection free.

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