Scleroderma is an autoimmune disorder, in which the immune system starts to attack and damage healthy cells. The condition is typically associated with skin tightening and hardening.
Juvenile scleroderma is a rare type of scleroderma that occurs in children. It is generally localized and only affects the skin. In more severe cases, it can also affect the connective tissue, muscles, and bones, but unlike the more severe systemic scleroderma, it does not affect other organs such as the heart and the lungs.
The exact cause of juvenile scleroderma is unknown. Some children may be more susceptible based on genetic factors. The condition may also be triggered by environmental factors such as an injury to the skin, infections, or a drug or toxin. Juvenile scleroderma is more common in females than males.
Once the abnormal immune response has been triggered, it can cause inflammation and produce a wound response that results in excess production of a protein called collagen. This excess collagen results in fibrosis, or scarring, in the affected tissue, causing it to harden and stiffen.
The most common form is called morphea, which is normally mild. It appears as patches of skin on the arms, legs, or trunk that can become pale, dry, and hard.
Linear scleroderma normally causes streaks of affected skin over a leg or arm. As the skin tightens and hardens, it can reduce mobility and cause damage if it crosses a joint. If left untreated, it can also affect the development and growth of the limb, resulting in arms and legs of uneven size.
Scleroderma en coup de sabre is a term used to describe children with lesions on the head or face. Similar to linear scleroderma, this often appears as streaks of tight and hard skin on the scalp and forehead, but may also be present on the chin or lip.
Parry Romberg syndrome is very similar to scleroderma en coup de sabre, but it may also involve the tongue or an entire side of the face rather than just patches. This may result in severe long-term effects, as it can affect the growth and development of the bones of the face.
Symptoms of juvenile scleroderma vary based on the type and severity of the condition. A common symptom of juvenile scleroderma is Raynaud’s phenomenon, or painfully cold and blue-tinted extremities.
In less than 10 percent of cases, a child with juvenile scleroderma can also be diagnosed with systemic sclerosis. This can present as lesions on the fingers, hands, forearms, and face, as well as causing damage to the internal organs.
There are no specific tests to diagnose juvenile scleroderma. A diagnosis is usually reached based on the symptoms and an examination of the lesions. A skin biopsy, where a small section of the lesion is removed to be examined under a microscope, may be used to support the diagnosis.
The therapy required varies depending on the severity of the condition. In mild cases, the patient may only require topical treatment, such as calcipotriene, to control the inflammation and reduce the hardening of the skin. This can include moisturizers to soften and protect the skin.
Medications such as corticosteroids also can be used to reduce inflammation. However, because these can cause severe side effects, they must be carefully managed.
Immune system suppressants, such as methotrexate, may be used off-label in more severe cases, especially where there is a risk of permanent damage. However, the use of these medications is still controversial, and they may cause severe side effects. Other immune-modulating therapies include tacrolimus, pimecrolimus, and imiquimod.
Physiotherapy may be recommended to improve physical ability and reduce stiffness.
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