© Georg Thieme Verlag KG Stuttgart · New York
A fulminant course of Cronkhite-Canada syndrome
17 December 2010 (online)
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.
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  . Cronkhite-Canada syndrome should be considered in all patients with gastrointestinal polyposis together with emergent alopecia and/or nail dystrophy .
Competing interests: None
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J. Martinek, MD, PhD
Department of Internal Medicine
Central Military Hospital
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