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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Limited scleroderma


Other Names for this Disease

  • Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
  • Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
  • CREST syndrome
  • Limited cutaneous systemic scleroderma
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Cause

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What causes CREST syndrome?

In people with CREST syndrome, the immune system appears to stimulate cells called fibroblasts to produce excess amounts of collagen. Normally, fibroblasts synthesize collagen to help heal wounds, but in this case, the protein is produced even when it's not needed, forming thick bands of connective tissue around the cells of the skin, blood vessels and in some cases, the internal organs.[1]

Although an abnormal immune system response and the resulting production of excess collagen appears to be the main cause of limited scleroderma, researchers suspect that other factors may play a role, including: genetic factors, pregnancy, hormones, and environmental factors.[1]

Last updated: 12/7/2011

References
  1. Mayo Clinic Staff. Limited Scleroderma (CREST syndrome). MayoClinic.com. 2011; http://www.mayoclinic.com/health/crest-syndrome/DS00580. Accessed 12/7/2011.


Other Names for this Disease
  • Calcinosis, Raynaud's phenomenon, Esophageal dismobility, Sclerodactyly, Telangiectasia syndrome
  • Calcinosis-Raynaud phenomenon-sclerodactyly-telangiectasia
  • CREST syndrome
  • Limited cutaneous systemic scleroderma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.