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Information

Two distinct subsets can be identified:

  1. Diffuse cutaneous SSc: rapid development of symmetric skin thickening of proximal and distal extremity, face, and trunk. At high risk for development of visceral disease early in course.
  2. Limited cutaneous SSc: often have long-standing Raynaud's phenomenon before other features appear; skin involvement limited to fingers (sclerodactyly), extremity distal to elbows, and face; generally associated with better prognosis but can be associated with pulmonary arterial hypertension; a subset of limited SSc has features of CREST syndrome (calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, telangiectasias).

Outline

Section 12. Allergy, Clinical Immunology, and Rheumatology