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| Fig. 22.7 CREST syndrome. A, Firm, tender, whitish papules of calcinosis cutis on the elbow. B, Sclerodactyly and telangiectasia on the fingers. |
• Calcinosis cutis
• Raynaud’s phenomenon
• Esophageal dysfunction (or dysmotility)
• Sclerodactyly
• Telangiectasia
CREST syndrome, or Thibierge-Weissenbach syndrome, is generally considered a type of limited systemic sclerosis. In addition to the cutaneous changes of calcinosis cutis, Raynaud’s phenomenon, sclerodactyly, and telangiectasia, these patients often develop hyperpigmentation, particularly in sun-exposed areas (Fig. 22-7). Most patients with the CREST syndrome have circulating antibodies to centromeres, called anticentromere antibodies.
