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.

Keywords: Corticosteroids; Cronkhite-Canada syndrome; Nonhereditary polyposis; Remission.

Fig. 1

Initial esophago-gastro-duodenoscopy. Diffuse sessile polypoid lesions in the stomach were seen (a: esophagus, b: antrum, c: great curvature, d: anterior wall of the upper part of stomach angle, e: cardia, and f: duodenum).

Fig. 2

Colonoscopy before treatment (a: transverse colon, b: terminal ileum, c: terminal ileum stained with indigo carmine). Polypoid lesions were observed only in the terminal ileum.

Fig. 3

Pathological findings of gastric polypoid lesions. Pathology revealed hyperplastic polyp without focal dilated cystic glands.

Fig. 4

Pathological findings of polypoid lesions in the terminal ileum. Similar findings to gastric lesions were seen.

Fig. 5

Esophago-gastro-duodenoscopy 1 year after treatment with corticosteroids. The size and the number of gastric polypoid lesions decreased (a: antrum, b: great curvature, c: cardia, and, d: anterior wall of the upper part of stomach angle). Some of the polypoid lesions disappeared and showed scar formations (e).