Cronkhite-Canada Syndrome Successfully Treated by Corticosteroids before Presenting Typical Ectodermal Symptoms
Open Access
- 1 September 2020
- journal article
- research article
- Published by S. Karger AG in Case Reports in Gastroenterology
- Vol. 14 (3), 561-569
- https://doi.org/10.1159/000510920
Abstract
Cronkhite-Canada syndrome (CCS) is a rare disease characterized by diffuse gastrointestinal polyposis with chronic diarrhea and ectodermal change, but its etiology is unknown. We present a case at the age of 26 years complaining of epigastralgia and weight loss. Endoscopic examination revealed extensive diffuse polypoid lesions of the stomach and the terminal ileum, all of which showed hyperplastic polyps pathologically. There were no polypoid lesions in his colon. He has no family history of diffuse gastrointestinal polyposis. Diffuse gastrointestinal hyperplastic polyposis without any hereditary association led us to suspect this case as CCS although he did not show chronic diarrhea and any ectodermal symptoms such as onychodystrophy, alopecia, and hyperpigmentation. After initiation of a corticosteroid therapy, his epigastralgia disappeared and he gained appetite and weight, accompanied by normalization of serum albumin levels. Endoscopic examination 1 year after initiation of corticosteroid therapy revealed a decrease in the number of gastric polyposis and those inflammations. This rare young case may suggest that early therapeutic intervention with corticosteroids could improve the prognosis of CCS, preventing not only malnutrition but also appearance of several ectodermal symptoms.This publication has 10 references indexed in Scilit:
- A Case of Adolescent Cronkhite-Canada SyndromeGastroenterology Research, 2018
- Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reportsBMC Gastroenterology, 2016
- Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhoea in a Young ManCase Reports in Medicine, 2016
- Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide surveyThe Esophagus, 2015
- Cronkhite-Canada syndrome: Report of six cases and review of literatureWorld Journal of Gastroenterology, 2014
- Hamartomatous Polyposis SyndromesSurgical Clinics of North America, 2008
- The non‐inherited gastrointestinal polyposis syndromesAlimentary Pharmacology & Therapeutics, 2002
- [Cronkhite-Canada syndrome: observations about treatment, course and prognosis of 123 cases reported in Japan].1988
- Cronkite-Canada Syndrome: Gastrointestinal Pathophysiology and MorphologyGastroenterology, 1972
- Generalized Gastrointestinal PolyposisThe New England Journal of Medicine, 1955