Cronkhite-Canada Syndrome Showing Good Early Response to Steroid Treatment

Woohee Cho; Kwangwoo Nam; Ki Bae Bang; Hyun Deok Shin; Jeong Eun Shin

doi:10.4166/kjg.2018.71.4.239

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Cho, Nam, Bang, Shin, and Shin: Cronkhite-Canada Syndrome Showing Good Early Response to Steroid Treatment

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Korean J Gastroenterol 2018;71(4):239-243.

Published online: 4 October 2018

DOI: https://doi.org/10.4166/kjg.2018.71.4.239

Cronkhite-Canada Syndrome Showing Good Early Response to Steroid Treatment

Woohee Cho, Kwangwoo Nam, Ki Bae Bang, Hyun Deok Shin, Jeong Eun Shin

Department of Internal Medicine, Dankook University College of Medicine, Cheonan, Korea

Correspondence to: Kwangwoo Nam, Department of Internal Medicine, Dankook University Hospital, 201 Manghyang-ro, Dongnam-gu, Cheonan 31116, Korea. Tel: +82-41-550-7630, Fax: +82-41-550-7050, E-mail: nambag1108@gmail.com

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

References

1. Cronkhite LW Jr, Canada WJ. Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. New Engl J Med. 1955; 252:1011–1015.

2. Watanabe C, Komoto S, Tomita K, et al. Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite-Canada syndrome: a Japanese nationwide survey. J Gastroenterol. 2016; 51:327–336.

3. Wen XH, Wang L, Wang YX, Qian JM. Cronkhite-Canada syndrome: report of six cases and review of literature. World J Gastroenterol. 2014; 20:7518–7522.

4. Kang DU, Yang DH, Choi Y, et al. A case of Cronkhite-Canada syndrome showing spontaneous remission. Intest Res. 2013; 11:317–322.

5. Yun SH, Cho JW, Kim JW, et al. Cronkhite-Canada syndrome associated with serrated adenoma and malignant polyp: a case report and a literature review of 13 Cronkhite-Canada syndrome cases in Korea. Clin Endosc. 2013; 46:301–305.

6. Daniel ES, Ludwig SL, Lewin KJ, Ruprecht RM, Rajacich GM, Schwabe AD. The Cronkhite-Canada syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine (Baltimore). 1982; 61:293–309.

7. Sweetser S, Ahlquist DA, Osborn NK, et al. Clinicopathologic features and treatment outcomes in Cronkhite-Canada syndrome: support for autoimmunity. Dig Dis Sci. 2012; 57:496–502.

Fig. 1.

Clinical manifestation of the patient at the initial presentation. (A) Hair loss. (B) Skin pigmentation and nail dystrophy. (C) Tongue swelling and mucosal atrophy.

Fig. 2.

Initial upper and lower endoscopic findings. Numerous variable-sized sessile polyps with mucosal edema and hyperemia were observed in the (A, B) stomach, and (C, D) colon.

Fig. 3.

Histologic findings. (A) Dilatation of gland, and inflammatory cell infiltration in gastric mucosa (H&E, ×40). (B) Dilatation of gland, cystic dilatation of crypt, and edema of lamina propria in colonic mucosa (H&E, ×40).

Fig. 4.

Follow-up upper and lower endoscopic findings. Marked regression of polyposis was observed in the (A, B) stomach, and (C, D) colon.

Fig. 5.

Clinical improvement of the patient at 5 months after steroid administration. Recovery from (A) hair loss and (B) skin pigmentation and nail dystrophy.

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