Cronkhite-Canada syndrome with esophagus involvement and six-year follow-up: A case report

Abstract

Background: Cronkhite-Canada syndrome (CCS) is a rare, noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities. CCS polyps are distributed through the whole digestive tract, and they are common in the stomach and colon but very uncommon in the esophagus.

Case summary: Here, we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea, alopecia, and loss of his fingernails. Laboratory data indicated anemia, hypoalbuminemia, hypocalcemia, hypokalemia, and positive fecal occult blood. Endoscopy showed numerous polyps scattered throughout the digestive tract, including the esophagus. He was treated with nutritional support and glucocorticoids with remission of his symptoms.

Conclusion: Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms. Treatment should be individualized for each patient according to their therapy response. Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.

Keywords: Case report; Cronkhite-Canada syndrome; Gastrointestinal polyposis; Hormonal therapy; Prognosis.

Figure 1

Clinical presence of ectodermal abnormalities. A: Alopecia; B: Nail dystrophy.

Figure 2

Hyperpigmentation of the skin. A and B: It was most evident on his face (A) and bilateral upper extremities (B).

Figure 3

Endoscopy results. A: Granular apophyses below the dentate line were observed by the endoscopist, although they were not so obvious; B and C: Gastroscopy showed multiple polyps hyperplasia of the stomach and the duodenum; D-F: Colonoscopy showed that mucosa erosion and multiple apophyses with the appearance of polypuses were observed in the ileocecal valve, and numerous polyps were found in the whole colon; G-I: The endoscopic findings showed no improvement at 6-year follow-up.

Figure 4

Histological examination of biopsy specimens obtained from duodenal bulb and the colon. A: Duodenal bulb; B: The colon. Haematoxylin and eosin staining (× 10).

Figure 5

Improvement of the clinical symptoms. A-E: Hyperpigmentation of the skin, nail dystrophy, and alopecia were relieved at one-month (A), five months (B), and four years (C-E) follow-up.