<Sclerodermia> Is a Greek word that can be translated as "leather hard". The skin becomes translucent and hard. A number of diseases are characterized hardening of the skin. Some are more or less limited to the skin (localized scleroderma), and can appear in patches (morphea) or linear strips that can restrict the movement of the joints and cause deformation of the same (linear scleroderma, also known as linear morphea).

Sometimes linear scleroderma can affect only the face and scalp, this is called "scleroderma en coup de saber". Onset, before the part becomes hard and there may be a translucent purple edge around the area of ​​affected skin. Often there is loss of fat tissue under the skin and slower growth of muscle tissue and underlying bone.

A minority of children with scleroderma have, in addition to hardening of the epidermis, the involvement of other parts of the body (in this case is called systemic sclerosis).

In systemic sclerosis, the involvement of internal organs can lead to symptoms such as difficulty swallowing, often with heartburn. Sometimes the heart, lungs or kidneys may be affected by this are carried out tests and specific treatments. In systemic sclerosis there are often problems with circulation (Raynaud's phenomenon).

How common is it?

Scleroderma is a rare disease. Estimates of the frequency indicates a value not exceeding 3 new cases per 100,000 inhabitants per year (estimates in England).

Localized scleroderma is the most common form in children and is more common in girls. Less than 10% of children with systemic sclerosis develop scleroderma.

Scleroderma affects individuals of all ethnic groups, they are not investigations were performed in different ethnic groups. Systemic sclerosis, which can occur at any age, has the same incidence female / male before puberty, after puberty occurs predominantly in girls.

What are the causes of the disease?

  Scleroderma is an inflammatory disease, but the cause of the inflammation has not been discovered yet. This is probably an autoimmune disease, which means that the sick child's immune system reacts against itself. In systemic sclerosis are also affected other organs of the body. Inflammation causes swelling (swelling) and heat, followed by a 'excessive production of fibrous tissue.

The cause is unknown, possible triggers, direct trauma to the skin in cases of localized scleroderma, and canola oil in systemic sclerosis.

Is it inherited?

No, there is no evidence that scleroderma is a genetic disease, although there are some rare reports of cases in one family. Studies remain to be done on a significant number of cases. In studies involving adults, appears to be a correlation between genes associated with other autoimmune diseases.

Can it be prevented?

No, there is no type of prevention.

And 'contagious?

No. Some infections could possibly unleash the debut, but the disease itself is not contagious and affected children do not need to be isolated from others.

A) SCLERODERMA LOCALIZED

How is localized scleroderma diagnosis ta?

An indication of localized scleroderma is the appearance of hardened skin areas. Often early in these areas are outlined in red or purple, a sign of skin inflammation.

In advanced stages, the skin turns brown and then white to white-skinned ethnicities. In people of color, this may look like a bruise would later become white, is the first symptom that suggests the child or parent.

The diagnosis is made on the basis of the characteristic appearance of the skin. Often made the test that measures the heat of the skin (thermography). A magnetic resonance imaging may be useful to monitor the underlying tissue, muscle and bone. This is particularly useful for "scleroderma en coup de saber" that affects the face and skull. Blood tests are usually normal. In localized scleroderma usually there is a serious internal organ involvement.
 

What is the treatment for localized scleroderma?

Treatment is aimed at stopping the inflammation as quickly as possible. The therapy has little effect on the fibrous tissue that has formed. Once inflammation decreases the body is able to absorb partially the fibrous tissue and the skin returns to soft.

For the form of localized scleroderma often do not need any therapy, as it is sometimes necessary to use steroids and methotrexate. There are no studies that have clearly demonstrated the effectiveness of therapy. The drugs must be administered under medical supervision and prescribed by a pediatric rheumatologist and / or dermatologist.
In the case of morphea, it is important to an assessment by the physician's level of inflammation of the tissues beneath. Blood tests for im arcatori inflammation are usually normal. Drug therapy varies from no treatment, use of steroids and methotrexate. The process
often resolves spontaneously over a period ranging from several months to a year, but may recur.
In some cases we may use the limited use of steroid cream (2 weeks)

In linear scleroderma, however, requires a more aggressive treatment when they are
it involved essuti underlying muscle and bone. If untreated, the bone stops growing and leads to a shortening of the arms and legs, or prevents the growth of a hand for example.
There is loss of elasticity of the muscle tissues. Moreover, the hardened skin over a joint causes stiffness and deformity of the joints. Thermography measures the 'inflammation and can be a tool to assess the state of inflammation during and after treatment.

The anti-inflammatory treatment is the administration of steroids and methotrexate. At first, the steroids may be administered as an intravenous infusion. These treatments must be subject to the supervision and prescription of a pediatric rheumatologist and / or dermatologist with a specific interest in these diseases.

The linear scleroderma requires more aggressive therapy. In this form also the physical therapy is important. When the skin affected is located over a joint is indicated and where necessary to stretch deep connective tissue massage.

In the case where one leg is affected this may be of different length than the other and cause lameness, increasing the strain on the back, hips and knees. This can be avoided by using an upside to the shoes.

The Skin Camouflage "Camouflage" can help, especially if the face shows cracks
unsightly they can be corrected. In patients with white skin, the skin should be protected
from the sun creams with specific so that the part affected by morphea (which does not tans and not
darkens) is not too obvious.

B) SYSTEMIC SCLEROSIS

How is it diagnosed? What are the main symptoms?

The early signs of disease are the color changes in fingers and toes moving from hot to cold (Raynaud's phenomenon), the presence of chilblains and ulcers on the fingertips. The skin of the fingers and toes often rapidly thickens and becomes translucent, like that of the nose. The skin induration may cover the entire body. At the beginning of the disease may also have swollen fingers (swollen) and digital ulcers. It 's important to perform specific tests to verify the involvement of internal organs. The child must be taken into care by a pediatric rheumatologist.

What is the treatment of systemic sclerosis in children?

Varies depending on the organ involved. Methotrexate for the treatment of the epidermis has proven efficacy. For swollen joints and elevated markers of inflammation resulting from blood tests are used both steroids and drugs such as methotrexate.
In case of Raynaud's phenomenon is necessary to promote the circulation through the heat; in order to prevent skin ulcers are sometimes necessary vasodilators. In the case of pulmonary or renal involvement are often used to cyclophosphamide.

The choice of therapy should be done by a pediatric rheumatologist expert in scleroderma with the collaboration of other specialists such as cardiologists and nephrologists.

Physiotherapy is used when the disease to maintain the functional capacity of the joints and chest.

What tests are needed periodically?

The frequency of checks depends on the stage of the disease and may be more frequent at the beginning. The objective is to adapt the therapy to the developments gradually monitored in the conditions of the individual child. Likewise, periodic checks are necessary to detect possible side effects related to therapy.

In localized scleroderma, pediatricians, dermatologists sometimes have carried out periodic checks on children rheumatology. Because scleroderma can be involved in major internal organs (lungs, gastrointestinal tract, heart, kidneys) is crucial to make periodic checks to detect any abnormalities early. Controls tests verify the development of inflammation and indicate the autoimmune process.

How long will the disease?

In the case of linear scleroderma disease duration is usually limited to several years. Often the hardening of the skin cease after two years into the disease. Sometimes you need to heal about 5-6 years. Some patches may become more apparent after the end of the inflammatory process or disease can result in uneven growth among the body parts involved and the healthy ones.

Systemic sclerosis is a long-term disease that can last a lifetime.

What is the long-term evolution of the disease?

•   Morphea usually leaves only cosmetic blemishes on the skin.
• The linear scleroderma might cause the children serious problems due to muscle atrophy, alteration of bone growth and joint deformities. Recent treatments have minimized these effects in most cases.
• Fortunately, compared to the adult form, systemic sclerosis in children has a more favorable prognosis. However, it is always a serious and potentially fatal disease that requires targeted therapies and medical experts. Generally the disease stabilizes after a certain number of years but the long-term monitoring even when they become adults it is important to control the development of complications.

And 'possible to recover completely?

Children with localized scleroderma heal. After some time even hardened skin may soften and return to normal.

Recovery from systemic sclerosis is much less likely, but you can achieve significant improvements or at least stabilize her, especially in adulthood.

Text translation Italian League Systemic Sclerosis Onlus 22/11/2011

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Source: Childhood Onset Scleroderma

© 2011 The Scleroderma Society - Revised / January 2011

PO Box 581 - Chichester - PO19 9EW

info@sclerodermasociety.co.uk ; www.sclerodermasociety.co.uk