Cronkhite-Canada syndrome presenting as eosinophilic gastroenteritis

Abstract

Cronkhite-Canada syndrome (CCS), first described in 1955, is a rare clinical syndrome of unknown etiology. CCS is diagnosed clinically, and the presenting symptoms include alopecia, cutaneous hyperpigmentation, gastrointestinal polyposis, and onychodystrophy, often accompanied by diarrhea, weight loss, and abdominal pain. We describe a unique case of CCS that presented with eosinophilic infiltrate on gastric and duodenal biopsies and review the literature pertaining to this rare syndrome.

Figure 1

Esophagogastroduodenoscopy results. (a) Endoscopic view showing thickened hypertrophic rugal folds in the body of the stomach. (b) The mucosa was erythematous and edematous on close inspection.

Figure 2

(a) Low-power view demonstrating blunting of the villi.(b) Medium-power and (c) high-power views illustrating the prominent eosinophilia in the lamina propria of the duodenal mucosa.

Figure 3

Cutaneous symptoms. (a) Diffuse thinning of the hair began shortly after the cholecystectomy. (b) The patient's fingers and toes were affected by onychodystrophy (arrowheads).

Figure 4

A small bowel radiograph with contrast revealed polyposis in the proximal small bowel (arrow).

Figure 5

Results of an upper endoscopic exam conducted 6 weeks after the initial study. (a) Polyps were scattered throughout the colon. (b) Atrophic gastric mucosa was present with retention-like polyps.