Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 16, 2021; 9(14): 3466-3471
Published online May 16, 2021. doi: 10.12998/wjcc.v9.i14.3466
Cronkhite-Canada syndrome with steroid dependency: A case report
Dan Jiang, Guo-Du Tang, Ming-Yu Lai, Zhen-Ning Huang, Zhi-Hai Liang
Dan Jiang, Guo-Du Tang, Ming-Yu Lai, Zhen-Ning Huang, Zhi-Hai Liang, Department of Gastroenterology, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China
Author contributions: Jiang D collected basic patient information and imaging data and wrote the main content of the manuscript; Tang GD reviewed the literature and made the diagnosis; Lai MY and Huang ZN were responsible for the analysis and interpretation of data; Liang ZH was responsible for the conception and design of the study and the final approval of the version of the article to be published; All authors have read and approve the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Zhi-Hai Liang, MD, Professor, Department of Gastroenterology, The First Affiliated Hospital of Guangxi Medical University, No. 6 Shuangyong Road, Nanning 530021, Guangxi Zhuang Autonomous Region, China. lzh@stu.gxmu.edu.cn
Received: December 15, 2020
Peer-review started: December 15, 2020
First decision: January 17, 2021
Revised: January 27, 2021
Accepted: March 15, 2021
Article in press: March 15, 2021
Published online: May 16, 2021
Processing time: 135 Days and 5.1 Hours
Abstract
BACKGROUND

Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disease characterized by chronic diarrhoea, diffuse gastrointestinal polyposis and ectodermal manifestations. The lethality of CCS can be up to 50% if it is untreated or if treatment is delayed or inadequate. More than 35% of the patients do not achieve long-term clinical remission after corticosteroid administration, with relapse occurring during or after the cessation of glucocorticoid use. The optimal strategy of maintenance therapy of this disease is controversial.

CASE SUMMARY

A 47-year-old man presented to the hospital with a 3-mo history of frequent watery diarrhoea, accompanied by macular skin pigmentation that included the palms and soles, and onychodystrophy of the fingernails and toenails. Gastroscopy and colonoscopy revealed numerous polyps in the stomach and colon. After other possibilities were ruled out by a series of examinations, CCS was diagnosed and treated with prednisone. The patient took prednisone for more than 1 year before achieving complete resolution of his symptoms and endoscopic findings. The patient was then given prednisone 5 mg/d for 6 mo of maintenance therapy. With clinical improvement and polyp regression, prednisone was discontinued. Eight mo after the discontinuation of prednisone, the diarrhoea and gastrointestinal polyps relapsed. Therefore, the patient was given the same dose of prednisone, and complete remission was achieved again.

CONCLUSION

It is necessary to extend the duration of prednisone maintenance therapy for CCS. Prednisone is still effective when readministered after relapse. Surveillance endoscopy at intervals of 1 year or less is recommended to assess mucosal disease activity.

Keywords: Cronkhite-Canada syndrome; Endoscopy; Prednisone; Relapse; Maintenance therapy; Case report

Core Tip: Cronkhite-Canada syndrome (CCS) is a rare gastrointestinal polyposis syndrome. Here, we report a case of CCS that has been followed for almost 4 years. The patient was treated with prednisone. After he discontinued prednisone, his clinical and endoscopic manifestations relapsed. The patient was given prednisone again, and it was effective in bringing about a second remission. It is necessary to extend the duration of prednisone maintenance therapy for CCS. Surveillance endoscopy at intervals of 1 year or less is recommended to assess mucosal disease activity.