CREST syndrome (limited systemic sclerosis)

CREST syndrome is the limited cutaneous subtype of systemic sclerosis, named for its classic features of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, though the full expression of all five is uncommon and tends to develop gradually. The same immune-driven fibrotic process seen in systemic sclerosis is at work, and it is associated with the anti-centromere antibody more often than the diffuse form.

Skin involvement stays confined to the fingers, hands, and forearms, sometimes with the feet and lower legs, and cohort studies suggest limited cutaneous disease is the more common of the two systemic sclerosis subtypes. Research describes limited disease as generally following a more favorable course than diffuse systemic sclerosis, though it carries its own risk of pulmonary hypertension over time.

Common signs

Calcium deposits under the skin (calcinosis), Raynaud's, swallowing difficulty from esophagus involvement, tightening of the skin on the fingers (sclerodactyly), and small red spots on the skin (telangiectasia). Skin involvement is typically more limited than in diffuse scleroderma.

This is a plain-language summary for orientation, not a diagnosis. Mechanisms are described as current understanding, not settled fact. Work with your own clinician for your care.