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Scleroderma

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Also known as: Systemic Scleroderma; Systemic Sclerosis; Localized Scleroderma; Morphea; Linear Scleroderma; Limited Cutaneous Scleroderma; CREST; Diffuse Cutaneous Scleroderma; SSc; Circumscribed Scleroderma; Dermatosclerosis

What is scleroderma?

Scleroderma is a complex, multisystem disease that often progressively affects the skin, blood vessels, lungs, gastrointestinal tract, kidneys, heart, and musculoskeletal tissues. Scleroderma is inflammatory and has an autoimmune component, although the exact cause is not yet known. Individuals with scleroderma overproduce collagen, a fibrous protein that makes up connective tissue including skin, resulting in symptoms that range from cosmetic problems to tissue damage to life-threatening organ dysfunction.

Scleroderma is relatively rare and its prevalence is somewhat difficult to determine. It has been estimated that it may affect as many as 250 out of every million American adults. Scleroderma can affect anyone at any age, including children, but most cases are seen in those who are 30 to 50 years old. Women are affected four times more often than men are.

Although a predisposition is not directly inherited, there are some increased frequencies of scleroderma tied to ethnicity, such as the Choctaw Native Americans in Oklahoma, among whom scleroderma is a hundred times more common than seen in the general population. Localized scleroderma is more common in those of European descent, while systemic scleroderma affects more women of African descent.

Scleroderma has been associated with exposures to chemicals such as vinyl chloride, trichloroethylene, benzene, epoxy resins, silica dust, certain drugs, and contaminated rapeseed oil, but scleroderma itself has not been directly tied to an environmental trigger.

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