Cronkhite-Canada syndrome

Thomas G. Cotter, Badr F. Al Bawardy, Seth Sweetser

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Cronkhite-Canada syndrome (CCS) is a noninherited condition, associated with high morbidity, and characterized by gastrointestinal inflammatory polyposis, alopecia, onychodystrophy, hyperpigmentation, and diarrhea. The etiology of CCS is unknown, although evidence continues to emerge supporting an autoimmune basis. The diagnosis of CCS is clinicopathological and remains challenging. Corticosteroids and nutritional support remain the cornerstone of management, however, newer immunomodulatory agents have emerged as viable alternatives in recent years. The role of surgery remains limited to complications refractory to medical management. The question of whether polyps in CCS possess malignant potential remains controversial. Optimal cancer screening protocols have not been developed for CCS patients, owing to the rarity of the disease. However, careful surveillance is recommended. In this chapter, we provide a succinct overview of the history, etiology, risk factors, clinical manifestations, diagnosis, and management of CCS. The latest evidence on the pathogenesis of CCS, as well as novel treatment options, will be incorporated. We anticipate that this chapter will enhance the knowledge of clinicians on this rare condition, helping to facilitate an appropriate diagnosis and enhance the management of patients with CCS going forward.

Original languageEnglish (US)
Title of host publicationIntestinal Polyposis Syndromes: Diagnosis and Management
PublisherSpringer International Publishing
Pages101-116
Number of pages16
ISBN (Electronic)9783319281032
ISBN (Print)9783319281018
DOIs
StatePublished - Jan 1 2016

Keywords

  • Alopecia
  • Canada syndrome
  • Cronkhite
  • Cutaneous hyperpigmentation
  • Diarrhea
  • Dysgeusia
  • Gastrointestinal
  • IgG4 autoimmunity
  • Onychodystrophy
  • Polyposis syndromes

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Cotter, T. G., Al Bawardy, B. F., & Sweetser, S. (2016). Cronkhite-Canada syndrome. In Intestinal Polyposis Syndromes: Diagnosis and Management (pp. 101-116). Springer International Publishing. https://doi.org/10.1007/978-3-319-28103-2_8