Cronkhite-Canada Syndrome Successfully Treated by Corticosteroids before Presenting Typical Ectodermal Symptoms

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.

Cronkhite-Canada Syndrome: A Case Report

“… 6 A study reported that clinical manifestations of CCS can be divided into five types based on initial symptoms: i) diarrhoea; ii) taste abnormalities; iii) dry mouth; iv) hair loss and nail atrophy; and v) loss of appetite, malaise, nail atrophy, hair loss, and dysgeusia in the absence of diarrhoea. 4 The case in the study reported few consistent symptoms such as diarrhoea and ectodermal changes. Gastrointestinal polyposis is closely related to the malabsorption which induced these ectodermal changes as well as diarrhoea.…”

“… 11 A study revealed that appropriate medical therapy can alter the natural history of CCS. 4 Oral corticosteroid therapy (30-49 mg/day) appeared to be effective for active CCS. Its benefits, including clinical improvement and polyp regression, are usually apparent within 12 months.…”

“…With only over 500 cases being reported worldwide, Cronkhite-Canada Syndrome (CCS) has been considered a sporadic and rare clinical condition. 4 Clinical description of CCS was first made by Cronkhite and Canada in 1955. 5 Its occurrence is reported to be sporadic with no strong evidence to suggest a hereditary predisposition 4 except one reported in father and son of Indian descent.…”

“… 4 Clinical description of CCS was first made by Cronkhite and Canada in 1955. 5 Its occurrence is reported to be sporadic with no strong evidence to suggest a hereditary predisposition 4 except one reported in father and son of Indian descent. 6 The case in this study also did not report any hereditary history.…”

“… 6 The case in this study also did not report any hereditary history. Patients of European and Asian descent are affected by more 4 than 75% of cases from Japan. 6 As per our knowledge, the case presented in this study can be considered the first few cases in Nepal.…”