Systemic scleroderma is very unpredictable although most cases can be classified into one of four different general patterns of disease (see Classification). It is the most fatal of all the rheumatologic diseases. It occurs in around 30 persons per million population per year and there are an estimated 125,000 active cases in the United States and perhaps 2.5 million worldwide. This form of scleroderma can occur at any age but is most common in women of childbearing age. Systemic sclerosis (SSc, scleroderma) is the most serious form of the disease. In modern medical terminology, scleroderma has evolved to be “shorthand” for two main groups of illness – systemic sclerosis and localized scleroderma. There are many human diseases associated with tightening and thickening of the skin so there are many “sclerodermas”.
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