A fulminant course of Cronkhite-Canada syndrome

Inhaltsübersicht

A 57-year-old man suffered from watery diarrhea, weight loss, and abdominal pain for 4 months. Noticeable signs of nail dystrophy (onychomadesis of all nails) were present ([Fig. 1 a]) along with alopecia and cutaneous foci of hyperpigmentation ([Fig. 1 b]).

Fig. 1 Main physical findings. a All 20 nails with onychomadesis about 3 mm from the lunula. Several nail plates have been lost. b Hands with several small foci of skin hyperpigmentation.

Upper-gastrointestinal endoscopy revealed a large number of strawberry-like polyps of different size in the stomach ([Fig. 2]).

Fig. 2 Endoscopic findings in the stomach. a Polyps of gastric body and antrum. b Detailed view of gastric polyp using zoom and narrow-band imaging mode; note the strawberry-like appearance.

Colonoscopy revealed polyposis of the whole colon, including the rectum. The majority of the polyps had a strawberry-like, adenomatous, and hyperplastic appearance ([Fig. 3]).

Fig. 3 a, b Endoscopic view of colonic polyps.

Histologically, the majority of the polyps were juvenile-like with cystic dilatations of the glands and a benign mucinous epithelium. The glands were filled with a large amount of mucin ([Fig. 4]).

Fig. 4 Histology. a Juvenile-like polyp with enlarged mucin glands. b Detailed view of mucinous glandular epithelium.

Video 1 Endoscopy of the stomach.

Video 2 Endoscopy of the colon.

Some adenomatous polyps with low-grade dysplasia in the colon were also detected. A subsequent enteroscopy did not find polyps; however, edema and small indentation of the jejunum were present. Immunohistochemistry showed total alactasia and a strong positivity for tumor necrosis factor (TNF) in the macrophages and lymphocytes. Based on both the clinical and endoscopic picture, a diagnosis of Cronkhite-Canada syndrome was established.

Despite complex treatment, the clinical course was unfavourable. Since the clinical state of the patient did not improve, anti-TNF-α treatment was considered. TNF-α activity was examined in the small-intestinal mucosa, and the results showed a strong intracellular expression of TNF-α. Unfortunately, an experimental anti-TNF-α treatment could not be introduced, because of the rapid progression of the disease. The patient died 4 months after the diagnosis of Cronkhite-Canada syndrome was established.

Cronkhite-Canada syndrome is a rare non-hereditary polyposis syndrome of an unknown, possibly autoimmune etiology. The prognosis is poor and more than 50 % of patients die within 2 – 4 years [1] [2]. Cronkhite-Canada syndrome should be considered in all patients with gastrointestinal polyposis together with emergent alopecia and/or nail dystrophy [3].

Competing interests: None

Endoscopy_UCTN_Code_CCL_1AD_2AC

References

• 1 Tao K, Patel J K, Pampati V. Cronkhite-Canada syndrome: a case report and review of the literature.  Gastroenterol Res Pract. 2009;  Article ID 619378
• 2 Goto A. Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan.  Nippon Geka Hokan. 1995;  64 3-14
• 3 Kojima E, Tomita A, Matsumura M, Kinoshita I. Magnifying the endoscopic appearance of Cronkhite–Canada syndrome.  Gastrointest Endosc. 2009;  70 1242-1243