Systemic Sclerosis

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General Principles

Definition

Systemic sclerosis (scleroderma) is a systemic illness characterized by progressive fibrosis of the skin and visceral organs. The etiology is unknown, but many manifestations of scleroderma are secondary to vasculopathy.

Classification

  • Scleroderma can be subdivided based on anatomic skin distribution into localized scleroderma (morphea and linear scleroderma) and systemic sclerosis (diffuse cutaneous, limited cutaneous, and systemic sclerosis sine scleroderma). The limited cutaneous form involves the extremities distal to the knees and elbows as well as the face. Diffuse cutaneous scleroderma involves the skin of the proximal extremities and the trunk. Systemic sclerosis sine scleroderma affects the internal organs without skin involvement. The CREST syndrome is calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.
  • Nephrogenic systemic fibrosis is a potential complication of MRI with gadolinium contrast in patients with renal failure. It is associated with skin thickening and internal organ fibrosis resembling scleroderma, without Raynaud phenomenon or ANA positivity.

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General Principles

Definition

Systemic sclerosis (scleroderma) is a systemic illness characterized by progressive fibrosis of the skin and visceral organs. The etiology is unknown, but many manifestations of scleroderma are secondary to vasculopathy.

Classification

  • Scleroderma can be subdivided based on anatomic skin distribution into localized scleroderma (morphea and linear scleroderma) and systemic sclerosis (diffuse cutaneous, limited cutaneous, and systemic sclerosis sine scleroderma). The limited cutaneous form involves the extremities distal to the knees and elbows as well as the face. Diffuse cutaneous scleroderma involves the skin of the proximal extremities and the trunk. Systemic sclerosis sine scleroderma affects the internal organs without skin involvement. The CREST syndrome is calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.
  • Nephrogenic systemic fibrosis is a potential complication of MRI with gadolinium contrast in patients with renal failure. It is associated with skin thickening and internal organ fibrosis resembling scleroderma, without Raynaud phenomenon or ANA positivity.

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