PDF
ePub
Citation
Print
Abstract
Peutz-Jeghers syndrome is an autosomal dominant inherited disorder characterized by multiple gastrointestinal hamartomatous polyps and mucocutaneous pigmentation. Peutz-Jeghers syndrome has an incidence of approximately 1 in 25,000 to 300,000 births. Crohn's disease is a chronic inflammatory bowel disease that typically manifests as regional enteritis with its incidence ranging from 3.1 to 14.6 cases per 100,000 person-years in North America. Herein, we report a case of a 30-year-old male patient who had both Peutz-Jeghers syndrome and Crohn's disease. We believe that this is the first case in Korea and the second report in the English literatures on Peutz-Jeghers syndrome coincidentally accompanied by Crohn's disease.
References
1. Kopacova M, Tacheci I, Rejchrt S, Bures J. Peutz-Jeghers syndrome: diagnostic and therapeutic approach. World J Gastroenterol. 2009; 15:5397–5408.
2. Dormandy TL. Gastrointestinal polyposis with mucocutaneous-pigmentation (Peutz-Jeghers syndrome). N Engl J Med. 1957; 256:1093–1103.
3. Chang MS, Kim H, Kim WH, et al. Korean Study Group for Pathology of Digestive Disease. Gastrointestinal polyposisin Koreans: a nationwide survey of clinicopathologic analysis of 112 surgically resected cases. Korean J Pathol. 1998; 32:404–412.
4. Reid JD. Intestinal carcinoma in the Peutz-Jeghers syndrome. JAMA. 1974; 229:833–834.
5. Park JB, Yang SK, Myung SJ, et al. Clinical characteristics atdiagnosis and course of Korean patients with Crohn's disease. Korean J Gastroenterol. 2004; 43:8–17.
6. Yang SK. Current status and clinical characteristics of inflammatory bowel disease in Korea. Korean J Gastroenterol. 2002; 40:1–14.
7. Abraham C, Cho JH. Inflammatory bowel disease. N Engl J Med. 2009; 361:2066–2078.
8. Chung KM, Kim HS, Park SY, et al. The changes in incidence of Crohn's disease and intestinal tuberculosis in Korea. Korean J Gastroenterol. 2008; 52:351–358.
9. Posselt HG, Kohls C, Ball F, Bender SW. Familial occurrence of Peutz-Jeghers syndrome and Crohn disease with manifestation of both diseases in the same patient. Z Gastroenterol. 1985; 23:670–675.
Fig. 2.
Endoscopic findings. (A) Multiple polyps of variable sizes are observed in the stomach. Longitudinal ulcer (B) and hamartomatous polyps (C) are also found in the colon.
Fig. 3.
(A) Histologic examination of a resected polyp demonstrates branching bundles of smooth muscle fibers covered by hyperplastic mucosa compatible with hamartoma which is typical of a Peutz-Jeghers syndrome polyp (H&E, ×10). (B) Chronic inflammatory lesion with granuloma (arrow), suggestive Crohn's disease, is also observed (H&E, ×100).
XML Download