ABSTRACT
The hands shown here are those of a 59-year-old man who presents with heartburn and Raynaud’s phenomenon. Which of the following is not part of this syndrome?
a. Skin thickening of the trunk b. Sclerodactyly c. Esophageal dysmotility d. Telangiectasias e. Calcinosis cutis
• CREST syndrome is also known as “limited scleroderma”
• CREST syndrome is a multisystem disorder characterized by the following:
Calcinosis cutis Raynaud’s phenomenon Esophageal dysmotility Sclerodactyly Telangiectasias
• Skin involvement in CREST syndrome is limited to the extremities (whereas it is much more diffuse in scleroderma)
• Involvement of joints and tendons is rare
• Lung involvement (e.g., reduced diffusing capacity, pulmonary hypertension) occurs in 70% of patients
• Anticentromere antibody is found in 70% to 90% of patients, and antiscleroderma-70 antibody in 10%
• Patients with CREST are at increased risk for primary biliary cirrhosis
• Raynaud’s phenomenon usually occurs before the other changes
