Table 1:
Scleroderma Disease Classification
Localized Scleroderma
(Localized cutaneous fibrosis)
Systemic Scleroderma*
(Cutaneous and noncutaneous involvement)
  • En coup de sabre-linear lesions
    of scalp or face
  • Linear scleroderma-linear lesions seen in childhood
  • Limited/generalized morphea-circumscribed patches of sclerosis
  • Limited-sclerosis limited to hands, distal extremities, and CREST syndrome-a form of limited systemic scleroderma with calcinosis of the digits, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias
  • Diffuse-sclerosis of proximal extremities, trunk, and face
  • Scleroderma sine sclerosis-organ fibrosis; no skin lesions
* With varying degrees of internal organ involvement
Copyright 2004 The Cleveland Clinic Foundation

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