cutaneous systemic sclerosis

Clinical manifestations

• limited cutaneous systemic sclerosis with involvement restricted to face & extremities distal to the elbow
• tightness, thickening & swelling of digits extending proximal to the metacarpophalangeal joints
• sclerodactyly
• digital telangiectasias
• terminal digital pitting or ulceration
• Raynaud's phenomenon
• internal organ fibrosis generally not a feature
• patients may display features of CREST syndrome
  • calinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias)

Laboratory

• antinuclear antibody generally positive (see scleroderma)
• anti-centromere antibody (30%); 90% with CREST syndrome

Diagnostic procedures

• see scleroderma

Radiology

• chest X-ray
  • basilar interstitial pulmonary fibrosis
• barium swallow if dysphagia (see scleroderma)

Complications

• pulmonary hypertension
• scleroderma renal crisis
• also see scleroderma

Management

• see scleroderma

More general terms

• systemic sclerosis