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Introduction to Scleroderma
Scleroderma (Sc) describes a group of related connective tissue diseases involving skin, joints and internal organs. The cause is unknown. Scleroderma means hardening (fibrosis) of the skin that takes place because of a malfunction of the vascular and immune systems. Researchers believe that the immune system, which protects us against infection and cancer, becomes overactive in patients with scleroderma resulting in the overproduction of collagen (connective tissue or "scar" tissue) in the body. Scleroderma can be localized to the skin or may become systemic with excess collagen building up in and around various organs such as the esophagus, kidneys, lungs, gastrointestinal tract, heart skin, and peripheral nervous system. This can lead to many accompanying complications.
Many people with certain types of scleroderma experience a plateau in which the condition stabilizes, which is followed by a period of improvement and skin softening. The most critical period for systemic scleroderma is usually within the first two to five years of onset when there is the highest risk for rapid progression and the development of serious conditions.
Fortunately, scleroderma is relatively rare affecting between approximately 40,000 to 300,000 persons in the United States. There are approximately 19 new cases per million people diagnosed per year in the US. The disorder most commonly occurs in women between the ages of 20 and 40, however men and children can be affected as well. Women are affected approximately 4 times as frequently as men. The highest risk group is African-American women for whom the incidence is higher than that of Caucasian women. The disease is not contagious and is not thought to be inherited.
Most cases of scleroderma are seen initially by a dermatologist and, as the condition progresses, patients often are referred to the care of a rheumatologist. It is important for people diagnosed with scleroderma to be examined and followed by their doctor to evaluate the development of other autoimmune conditions or complications.
