Scleroderma, which literally means “hard skin,” describes a group of conditions that causes the skin to tighten and harden. There are two basic forms:
- Localized scleroderma. It is primarily a skin disease and is the type seen more commonly in children. Localized juvenile scleroderma can damage the skin, muscle, bones and joints, depending on the type. It is unlikely to cause damage to internal organs.
- Systemic sclerosis. This type affects the entire body. It causes internal organ damage and may be severe.
Juvenile scleroderma can occur at any age and in any race, but it is more common in girls. It is a rare disease. The exact number of patients affected is unknown.
Genetic factors may make a child more likely to developing localized scleroderma. Environmental factors, such as trauma, infections or drug or chemical exposure, may play a role in triggering the disease, but the relationships are unclear.
Localized scleroderma is an autoimmune disease in which the immune system mistakes the body’s own tissues as foreign invaders. In this case, the autoimmune reaction causes inflammation in the skin. It can trigger connective tissue cells to produce too much collagen. Excess collagen can lead to fibrosis, or a hardening and scarring of the tissues.
Symptoms depend on the type of scleroderma. In localized scleroderma, the skin may become thickened or thinned, lighter or darker, but is often smooth or shiny in appearance. Skin changes can occur anywhere, from the face, to the arms and legs or trunk of the body.
There are two types of localized scleroderma:
- Morphea. This type is characterized by one or more hard, oval-shaped, whitish or darkened patches of skin. Most often, there are no serious long-term consequences.
- Linear scleroderma. This form involves lines or streaks of thickened skin that form over an area of the body, such as an arm, leg or the head. It may may cause deep-tissue injury. Generally, only one arm or one leg is involved. Larger areas of linear scleroderma that extend over an arm or leg or cross a joint may cause permanent damage. Without proper treatment, permanent changes in the size of an arm or leg can result. "Scleroderma en coup de sabre" is the term applied when linear scleroderma crosses the head or face.
Systemic sclerosis is very rarely seen in children and it targets internal organs. Symptoms tend to affect the skin of the fingers, hands, forearms and face. It has more severe long-term effects.
Localized scleroderma usually is diagnosed based on the patient’s history and physical examination results. There are no specific laboratory studies to diagnose juvenile scleroderma, but tests often are done to evaluate the level of inflammation and to rule out other conditions. A skin biopsy may be done to confirm the diagnosis.
There is no known cure for juvenile scleroderma. The disease can go into remission, but the timing varies. Morphea lesions that do not extend into deeper tissues may go into remission within a few years, whereas linear scleroderma lesions—especially on the head—can remain active for many years.
Treatment of localized scleroderma is focused on controlling inflammation, because this decreases the risk of serious problems. It can be treated topically or systemically, depending on the extent of involvement.
For patients with mild disease, topical medications are used to control inflammation and soften the skin. These medications include corticosteroids, calcipotriene, tacrolimus, pimecrolimus and imiquimod. Moisturizers may help protect and soften the skin.
When large areas of an extremity are involved, or the disease is crossing a joint line and there is a significant risk of permanent damage, systemic medications that suppress the immune system are used. These medicines include methotrexate and corticosteroids, which are taken by mouth or given by infusion.
Most children with localized scleroderma don’t need to make major lifestyle changes, but disease self management is essential for all people living with a chronic disease. Physical activity, proper nutrition and skin care and following the doctor’s instructions should all be reinforced.