Cronkhite Canada syndrome

Abstract

A 59-year-old Chinese man presented with hypogeusesthesia, anorexia, progressive weight loss, complete dystrophy of all finger and toe nails, mild hyperpigmentation of the palms, alopecia, and chronic diarrhea. Colonoscopy revealed diffuse inflammation, ulceration and multiple polyps throughout the rectum and colon (A). At upper endoscopy, there was diffuse and extensive inflammation with multiple polyps in the body (B) and antrum of the stomach as well as the first and second parts of the duodenum. Barium contrast radiography of the small bowel revealed diffuse mucosal thickening of the jejunum and ileum. Histopathologic examination of a colon polyp (C; H&E, orig. mag. ×150) demonstrated marked edematous expansion of the lamina propria, which was infiltrated by eosinophils, lymphocytes, and plasma cells, and contained dilated cystic glands. Gastric and duodenal polyps exhibited similar histopathologic features. A diagnosis of Cronkhite Canada syndrome was made based on the presence of diffuse GI polyposis and associated features of nail dystrophy, skin hyperpigmentation, and alopecia. Treatment included total parenteral nutrition at home, which resulted in dramatic resolution of diarrhea, an increase in body weight, regrowth of normal nail plates, and fading of the palmar hyperpigmentation. Unfortunately, the patient died of sepsis and thrombosis of the superior vena cava 10 months after diagnosis.