Localised scleroderma

Two types exist: localised scleroderma which includes linear scleroderma, and systemic sclerosis.

In systemic scleroderma organs and skin are involved; in localised scleroderma the disease affects the skin and the tissues below. Arthritis and joint inflammation are common to both types.

The disease progression of localised scleroderma is limited to a few years and many children recover completely although skin pigmentation may persist. In severe cases, hardening of the skin can restrict muscle and bone development and when localised over a joint may result in arthritis of that joint.

• Changes in the colour of fingers and toes in cold temperatures.
• Hardening of the skin on fingers and toes that may become shiny. If untreated this shiny skin can spread over the body.
• Other skin changes such as dilated blood vessels, calcifications or deposits of calcium under the skin, as well as peeling and wasting of skin layers.
• Sore and swollen joints are common.
• Heartburn and difficulty swallowing can occur if the lining of the oesophagus is affected.
• Inflammation of the intestinal tract can cause bloating and poor digestion.
• Inflammation of the lung is frequent. In severe cases, membranes of the heart, kidney and other organs can occur.

There is no specific blood test for scleroderma. Diagnosis is made by physical examination and assessment of the symptoms. Laboratory tests are used to assess severity of inflammation and to eliminate other diseases.

Ongoing evaluation is important after diagnosis to assess efficacy of treatment.

In some cases, the inflammation may resolve by itself over a few years and does not require medication. In the majority of cases, corticosteroids are prescribed.

The main corticosteroids used in treating both types of scleroderma are:

• Methotrexate
• Mycophenolate
• Cyclophosphamide

In severe cases of systemic scleroderma autologous bone marrow transplantation is undertaken.

Physiotherapy

Physiotherapy is very important. It helps prevent tight skin from effecting the joints and muscles below and is a fundamental treatment in systemic scleroderma if the lungs and chest wall are affected.

Skin care

Massage and moisturising creams may be recommended to soften and relax tightened skin lesions. Cosmetics can help cover the skin with pigmentation damage, especially on the face.

 

If you are using the internet to search for information about you child’s disease, in addition to the excellent Australian resources listed, we recommend information provided by Paediatric Rheumatology InterNational Trials Organisation (PRINTO)

PRINTO is an international public network of academic and/or clinical centres engaged in the research and clinical care of children with paediatric rheumatic diseases. They collect a lot of information world-wide and so the information they provide is reliable and up-to-date.