Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Aug 16, 2016; 4(8): 248-252
Published online Aug 16, 2016. doi: 10.12998/wjcc.v4.i8.248
Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells
Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Ran An, Jian-Qiu Sheng
Ru-Ying Fan, Xiao-Wei Wang, Li-Jun Xue, Jian-Qiu Sheng, Department of Gastroenterology, Beijing Military General Hospital, Beijing 100700, China
Ran An, Department of Pathology, Beijing Military General Hospital, Beijing 100700, China
Author contributions: Fan RY designed, wrote and revised the paper; Wang XW and Xue LJ treated patient and collected clinical data from patient; An R responsible for pathologic diagnosis; and Sheng JQ responsible for endoscopic diagnosis.
Informed consent statement: The patient involved in this study gave his written informed consent authorizing use and disclosure of his protected health information.
Conflict-of-interest statement: All the authors have no conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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:
Correspondence to: Ru-Ying Fan, MD, Department of Gastroenterology, Beijing Military General Hospital, No. 5 Nanmencang, Dongcheng District, Beijing 100700, China.
Telephone: +86-10-66721168 Fax: +86-10-66721629
Received: April 23, 2016
Peer-review started: April 23, 2016
First decision: May 17, 2016
Revised: May 24, 2016
Accepted: June 14, 2016
Article in press: June 16, 2016
Published online: August 16, 2016

Cronkhite-Canada syndrome (CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin (30.1 g/L, range: 35.0-55.0 g/L), serum potassium (2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose (2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h (range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.

Keywords: Gastrointestinal polyposis, Cronkhite-Canada syndrome, IgG4 plasma cells, Autoimmune mechanism

Core tip: Cronkhite-Canada syndrome (CCS) is a non-hereditary condition characterized by gastrointestinal polyposis associated with diarrhea and epidermal manifestations. It is a rare but serious disease, early diagnosis can improve prognosis of the patients, but delay in diagnosis is common due to non-familiarity of its clinical manifestation. Here we report a case of a patient with CCS, in this report showed the patient’s clinical characteristics and response to treatment.