Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 28, 2024; 30(8): 984-990
Published online Feb 28, 2024. doi: 10.3748/wjg.v30.i8.984
Cronkhite-Canada syndrome with esophagus involvement and six-year follow-up: A case report
Yu-Chen Tang
Yu-Chen Tang, Department of Endocrinology, The First Affiliated Hospital of Ningbo University, Ningbo 315000, Zhejiang Province, China
Author contributions: Tang YC contributed to data collection, case analysis and manuscript writing.
Supported by the Medical Health Science and Technology Project of Zhejiang Province, No. 2022KY1109; the Natural Science Foundation of Ningbo, No. 2022J204; and Ningbo Key Clinical Specialty (Endocrinology), No. 2022-B07.
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 author declare that they have no conflict of interest to disclose.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yu-Chen Tang, MD, Attending Doctor, Department of Endocrinology, The First Affiliated Hospital of Ningbo University, No. 59 Liuting Street, Ningbo 315000, Zhejiang Province, China. fyytangyuchen@nbu.edu.cn
Received: September 28, 2023
Peer-review started: September 28, 2023
First decision: December 28, 2023
Revised: January 5, 2024
Accepted: January 25, 2024
Article in press: January 25, 2024
Published online: February 28, 2024
Abstract
BACKGROUND

Cronkhite-Canada syndrome (CCS) is a rare, noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities. CCS polyps are distributed through the whole digestive tract, and they are common in the stomach and colon but very uncommon in the esophagus.

CASE SUMMARY

Here, we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea, alopecia, and loss of his fingernails. Laboratory data indicated anemia, hypoalbuminemia, hypocalcemia, hypokalemia, and positive fecal occult blood. Endoscopy showed numerous polyps scattered throughout the digestive tract, including the esophagus. He was treated with nutritional support and glucocorticoids with remission of his symptoms.

CONCLUSION

Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms. Treatment should be individualized for each patient according to their therapy response. Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.

Keywords: Cronkhite-Canada syndrome, Gastrointestinal polyposis, Hormonal therapy, Prognosis, Case report

Core Tip: Cronkhite-Canada syndrome is a rare, noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities. We report a case of a 63-year-old man with numerous polyps scattered throughout the digestive tract, including the esophagus. Comprehensive corticosteroid treatment can result in partial or full remission of clinical symptoms.