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Juvenile Systemic Sclerosis

Practice Essentials

Systemic sclerosis (also scleroderma) is a chronic, autoimmune rheumatic disease affecting the skin and other organs. The hallmark of the disease is thickening/tightening of the skin and inflammation/scarring of various organs/body parts, leading to involvement of the lungs, kidneys, heart, intestinal system, and other areas. There is no cure for scleroderma but effective treatments for some features of the disease are available. The disease is relatively uncommon. About 75,000–100,000 people in the United States have the disease; most are women between the ages of 30 and 50 years. Twins and family members of those with scleroderma or other autoimmune connective tissue diseases, such as lupus, may have a slightly higher risk of developing scleroderma. Children can also get scleroderma, but the disease is different in children from that in adults. Although the underlying cause is unknown, research is shedding more light on the relationship between genes, the immune system and scleroderma. Research is also underway to find better treatments for scleroderma and, hopefully, one day a cure.

There are two main categories of scleroderma:

Localized scleroderma affects skin, underlying connective tissues to a greater or lesser extent. Discolored, thickened patches of skin, termed morphea, may be so subtle as to escape the examiner’s attention unless pointed out. When streaks/bands of thickened tissue involve a limb, it is termed linear scleroderma. Linear scleroderma involving the face, forehead, and scalp is termed “scleroderma en coup de sabre.” Diffuse cutaneous scleroderma without systemic involvement can occurr, but rarely.  

Systemic scleroderma (systemic sclerosis), which is the most serious form of the disease, affects skin, muscles, bones, joints, blood vessels, lungs, kidneys, heart, and virtually all organs. Progressive systemic sclerosis is a rare, inexorably progressive disease that leads to an early death.

There is no known cause. Genetic factors and the immune system appear to be important in disease development and progression. Although exposure to certain chemicals and drugs may play a role in some children developing scleroderma, the vast majority of patients do not have a history of exposure to any suspicious toxins. It should be noted that as with other rheumatic diseases of childhood, cases with features of two or more conditions is not uncommon. Most overlap cases have features of scleroderma and dermatomyositis. In some children it may take years before a definitive diagnosis is possible. 

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