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Miry N, Bennani A, Kharrasse G. A Rare Case of Cronkhite-Canada Syndrome in a Moroccan Female Patient. Cureus 2025; 17:e79977. [PMID: 40177436 PMCID: PMC11964408 DOI: 10.7759/cureus.79977] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/03/2025] [Indexed: 04/05/2025] Open
Abstract
Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disorder characterized by gastrointestinal polyps and ectodermal manifestations, such as alopecia and nail dystrophy. This report highlights the diagnostic and therapeutic challenges of CCS and contributes to the global understanding of the syndrome. A 50-year-old woman presented with persistent anorexia, fatigue, abdominal discomfort, alopecia, and onychodystrophy. Diagnostic evaluation revealed characteristic endoscopic findings of gastrointestinal polyps and histopathological features consistent with CCS. The patient was treated with glucocorticoids, resulting in clinical and nutritional improvement. Nutritional support and immunosuppressive agents may serve as adjunct therapies, but further research is needed to establish definitive guidelines. This case underscores the importance of thorough endoscopic and histopathological evaluations in rare syndromes like CCS. It also emphasizes the significance of early diagnosis and a multidisciplinary approach to disease management.
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Affiliation(s)
- Nadir Miry
- Department of Pathology, Mohammed VI University Hospital, Oujda, MAR
- Faculty of Medicine and Pharmacy of Oujda, Mohammed First University, Oujda, MAR
| | - Amal Bennani
- Department of Pathology, Mohammed VI University Hospital, Oujda, MAR
- Faculty of Medicine and Pharmacy of Oujda, Mohammed First University, Oujda, MAR
| | - Ghizlane Kharrasse
- Department of Gastroenterology and Hepatology, Mohammed VI University Hospital, Oujda, MAR
- Faculty of Medicine and Pharmacy of Oujda, Mohammed First University, Oujda, MAR
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Lu Y, Huang F, Wang Y, Zhou J, Zhao Q, Liu L. Clinical and Endoscopic Characteristics of Chinese Cronkhite-Canada Syndrome Patients: A Retrospective Study of 103 Cases. Dig Dis 2021; 39:488-495. [PMID: 33440392 DOI: 10.1159/000514354] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/23/2020] [Accepted: 01/11/2021] [Indexed: 02/02/2023]
Abstract
INTRODUCTION Cronkhite-Canada syndrome (CCS) is a rare non-inherited disease characterized by extensive gastrointestinal (GI) polyposis and ectodermal dysplasia. So far, most of CCS related literatures are published as single case report or reviewed with limited case numbers. Our study was to update the clinical and endoscopic characteristics of Chinese CCS patients. METHODS This retrospective study was conducted in 103 Chinese CCS patients (102 cases from literatures and 1 case from our department). Their clinical and endoscopic data were collected, and statistical analyses were performed. RESULTS (1) In Chinese population, people aged 50-70 years (62.62%) had a high incidence of CCS, and the ratio of male-to-female was 2.68:1. (2) The diverse range of GI manifestations was observed in all the patients, and almost all the patients had at least 1 symptom of ectodermal dysplasias. (3) All CCS patients presented multiple polyps in the GI tract except esophagus, and the size and appearance of polyps were diverse. Congestion, edema, and erosion were very common on the surface of polyps (96.83%) and the surrounding mucosa (85.71%). (4) The common pathological features of polyps were hyperplastic polyps (49.25%) and tubular adenomatous polyps (44.78%). The prevalence of cancer was 5.97% in Chinese CCS patients. CONCLUSIONS Middle-aged and elderly people are the high-risk group. Various GI symptoms are observed in Chinese patients; the typical endoscopic finding is multiple small sessile polyps. These GI polyps have a chance of malignant potential. Long-term endoscopic surveillance and follow-up are recommended for the Chinese CCS patients.
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Affiliation(s)
- Yuanyuan Lu
- Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, China.,Hubei Clinical Center & Key Laboratory of Intestinal & Colorectal Diseases, Wuhan, China
| | - Fengxing Huang
- Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, China.,Hubei Clinical Center & Key Laboratory of Intestinal & Colorectal Diseases, Wuhan, China
| | - Youwei Wang
- Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, China.,Hubei Clinical Center & Key Laboratory of Intestinal & Colorectal Diseases, Wuhan, China
| | - Juan Zhou
- Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, China.,Hubei Clinical Center & Key Laboratory of Intestinal & Colorectal Diseases, Wuhan, China
| | - Qiu Zhao
- Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, China.,Hubei Clinical Center & Key Laboratory of Intestinal & Colorectal Diseases, Wuhan, China
| | - Lan Liu
- Department of Gastroenterology, Zhongnan Hospital of Wuhan University, Wuhan, China.,Hubei Clinical Center & Key Laboratory of Intestinal & Colorectal Diseases, Wuhan, China
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Wu ZY, Sang LX, Chang B. Cronkhite-Canada syndrome: from clinical features to treatment. Gastroenterol Rep (Oxf) 2020; 8:333-342. [PMID: 33163187 PMCID: PMC7603875 DOI: 10.1093/gastro/goaa058] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2019] [Revised: 03/04/2020] [Accepted: 04/16/2020] [Indexed: 02/07/2023] Open
Abstract
Cronkhite–Canada syndrome (CCS) is a rare acquired polyposis with unknown etiology. To date, >500 cases have been reported worldwide. CCS is typically characterized by gastrointestinal symptoms, such as diarrhea and skin changes (e.g. alopecia, pigmentation, and nail atrophy). Endoscopic features include diffuse polyps throughout the entire gastrointestinal tract, except for the esophagus. Pathological types of polyps in CCS mainly include inflammatory, hyperplastic, hamartomatous, and adenomatous polyps. CCS can be complicated by many diseases and has a canceration tendency with a high mortality rate. Moreover, there is no uniform standard treatment for CCS. A review of the reported cases of CCS is presented herein, with the goal of improving our understanding of this disease.
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Affiliation(s)
- Ze-Yu Wu
- Department of Gastroenterology, First Hospital of China Medical University, Shenyang, Liaoning, P. R. China
| | - Li-Xuan Sang
- Department of Geriatrics, First Hospital of China Medical University, Shenyang, Liaoning, P. R. China
| | - Bing Chang
- Corresponding author. Department of Gastroenterology, First Affiliated Hospital of China Medical University, No. 155, Nanjing North Street, Shenyang, Liaoning 110001, P. R. China. Tel: +86-24-83283765; Fax: +86-24-83283764;
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Liu S, Ruan GC, You Y, Qian JM, Li J. A striking flail chest: a rare manifestation of intestinal disease. Intest Res 2018; 17:155-156. [PMID: 30541226 PMCID: PMC6361013 DOI: 10.5217/ir.2018.00132] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2018] [Accepted: 10/08/2018] [Indexed: 11/09/2022] Open
Affiliation(s)
- Shuang Liu
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Ge Chong Ruan
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Yan You
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jia Ming Qian
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Ji Li
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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Fan RY, Wang XW, Xue LJ, An R, Sheng JQ. Cronkhite-Canada syndrome polyps infiltrated with IgG4-positive plasma cells. World J Clin Cases 2016; 4:248-252. [PMID: 27574615 PMCID: PMC4983698 DOI: 10.12998/wjcc.v4.i8.248] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2016] [Revised: 05/24/2016] [Accepted: 06/16/2016] [Indexed: 02/05/2023] Open
Abstract
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.
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Traussnigg S, Dolak W, Trauner M, Kazemi-Shirazi L. Difficult case of Cronkhite-Canada syndrome with small intestinal bacterial overgrowth, Clostridium difficile infection and polymyalgia rheumatica. BMJ Case Rep 2016; 2016:bcr-2015-213465. [PMID: 26818813 DOI: 10.1136/bcr-2015-213465] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
A 64-year-old woman presented with heavy diarrhoea, nausea and weight loss accompanied by alopecia and dystrophic fingernails and toenails. The preceding diagnosis of an inflammatory bowel disease, a common pitfall, was excluded by endoscopic work up. Instead, Cronkhite-Canada syndrome (CCS), a rare polyposis condition, was identified as the reason for this almost pathognomonic combination of diagnostic findings including various polyps throughout the entire intestine and ectodermal abnormalities. This case exemplifies common risks and complications in terms of gastrointestinal malabsorption, infections and small intestinal bacterial overgrowth (SIBO), including its treatment as well as a hereto unreported association with polymyalgia rheumatica. In CCS, long-term immunosuppressive therapy and close endoscopic cancer screening of the patient is essential. The treatment of vitamin deficiency and recurring SIBO helps to reduce symptoms.
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Affiliation(s)
- Stefan Traussnigg
- Department of Internal Medicine III-Division of Gastroenterology and Hepatology, Medical University Vienna, Vienna, Austria
| | - Werner Dolak
- Department of Internal Medicine III-Division of Gastroenterology and Hepatology, Medical University Vienna, Vienna, Austria
| | - Michael Trauner
- Department of Internal Medicine III-Division of Gastroenterology and Hepatology, Medical University Vienna, Vienna, Austria
| | - Lili Kazemi-Shirazi
- Department of Internal Medicine III-Division of Gastroenterology and Hepatology, Medical University Vienna, Vienna, Austria
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