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Cronkhite-Canada syndrome with steroid dependency: A case report.

Dan Jiang1, Guo-Du Tang1, Ming-Yu Lai1

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Summary

Cronkhite-Canada syndrome (CCS) requires extended prednisone maintenance therapy. Prednisone remains effective even after relapse, and regular endoscopic surveillance is recommended for managing this rare gastrointestinal polyposis disease.

Keywords:
Case reportCronkhite-Canada syndromeEndoscopyMaintenance therapyPrednisoneRelapse

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Area of Science:

  • Gastroenterology
  • Rare Diseases
  • Clinical Medicine

Background:

  • Cronkhite-Canada syndrome (CCS) is a rare, nonhereditary disorder.
  • Characterized by chronic diarrhea, diffuse gastrointestinal polyposis, and ectodermal changes.
  • High lethality (up to 50%) if untreated; corticosteroid resistance is common.

Observation:

  • A 47-year-old male presented with a 3-month history of watery diarrhea, skin pigmentation, and onychodystrophy.
  • Endoscopic examination revealed extensive gastric and colonic polyposis.
  • Diagnosis of CCS was confirmed after excluding other conditions.

Findings:

  • Initial treatment with prednisone for over a year led to symptom resolution and polyp regression.
  • A subsequent 6-month maintenance therapy with prednisone was followed by discontinuation.
  • Relapse occurred 8 months post-discontinuation, with remission achieved again upon readministration of prednisone.

Implications:

  • Extended duration of prednisone maintenance therapy is necessary for CCS.
  • Prednisone is effective for managing CCS relapses.
  • Annual or more frequent endoscopic surveillance is crucial for monitoring mucosal activity.