Systemic sclerosis (often used interchangeably with scleroderma) is a rare chronic disease of connective tissue, characterized by progressive sclerosis (hardening) of tissues including the skin, vessels, internal organs, and joints.

  • The disease varies in its progression and involvement, ranging from localized cutanous lesions with slow progression, to generalized sclerosis of the skin and visceral organs, progressing rapidly.
  • The etiology is unknown, but it is thought that a dysregulation of the immune system activates fibroblasts which then deposit large amounts of collagen, leading to hardening (sclerosis).
  • More common in women.

Classification

Systemic sclerosis can be classified into 4 types:

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