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Oral Symptoms of Intractable Ulcerating Enterocolitis of Infancy and Differential Diagnosis: A Case Report

난치성 궤양성 소장결장염 영아의 구강 내 증상과 감별진단: 증례보고

  • Min, Hyoseon (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Choi, Hyungjun (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Jaeho (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Choi, Byungjai (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Hyoseol (Department of Pediatric Dentistry, College of Dentistry, Yonsei University)
  • 민효선 (연세대학교 치과대학 소아치과학교실) ;
  • 최형준 (연세대학교 치과대학 소아치과학교실) ;
  • 이제호 (연세대학교 치과대학 소아치과학교실) ;
  • 최병재 (연세대학교 치과대학 소아치과학교실) ;
  • 이효설 (연세대학교 치과대학 소아치과학교실)
  • Received : 2013.09.02
  • Accepted : 2013.12.10
  • Published : 2014.02.28

Abstract

Intractable ulcerating enterocolitis is an uncommon inflammatory bowel disease syndrome of neonatal onset first described in 1991. Intractable ulcerating enterocolitis usually presents in the neonate with a mouth ulceration and the subsequent development of perianal disease and colitis. In this case report, an infant, 18 days from birth, with ulcerative lesion on hard palate for systemic differential diagnosis about oral lesion is referred from the department depiatrics. At that time, there is no abnormality, except oral lesion-like Aphthous ulcer. The patient was discharged from pediatrics, but returned to the hospital 3weeks later with blood diarrhea. As a result of endoscopy, there were large ulcerating lesions and the patient was diagnosed intractable ulcerating enterocolitis. Early recognition of Intractable ulcerating enterocolitis appears to be beneficial because colectomy, as opposed to immunosuppression, appears to be effective in controlling disease symptoms and progression. Most of the infants who were affected intractable ulcerating enterocolitis were normal at birth and oral manifestation appeared earlier than others. So, it is very meaningful for dentists to know about Intractable ulcerating enterocolitis.

난치성 궤양성 소장결장염은 영아에게서 드물게 나타나는 만성 염증성 장 질환으로 1991년 처음으로 보고되었다. 난치성 궤양성 소장결장염은 주로 영아기에 구강 내 궤양에서 시작되어 항문주위 및 장 질환으로 진행되는 특징을 갖는다. 생후 18일 된 환아가 구강 내 궤양으로 감별할 수 있는 전신질환에 대해 알아보고자 본과로 의뢰 되었다. 당시 구강 내 궤양을 제외한 특이 사항은 없었으며 3주 뒤 혈변이 발생하였다. 대장 내시경 상에서 대장 내 거대 궤양이 발견되었으며 여러검사 결과 난치성 궤양성 소장결장염으로 진단되었다. 치과의사가 난치성 궤양성 소장결장염을 조기에 진단하는 것은 면역 억제제에 반응이 없는 본 질환에 대해 결장절제술을 신속하게 시행함으로써 증상의 완화와 질병의 진행을 막는데 효과적으로 대처할 수 있으므로 매우 중요하다. 또한 대부분의 난치성 궤양성 소장결장염 환아에게서 구강 내 궤양이 가장 먼저 발현되므로 이 질환의 증상과 특징에 대해 아는 것은 매우 의미 있을 것으로 사료되어 보고하는 바이다.

Keywords

References

  1. Sanderson I, Risdon R, Walker-Smith J: Intractable ulcerating enterocolitis of infancy. Archives of disease in childhood, 66:295-299, 1991. https://doi.org/10.1136/adc.66.3.295
  2. Jeong JY, Seo JK, KW P, Chi JG: A Case of "Intractable Ulcerating Enterocolitis"of Infants. Korean journal of Pediatrics, 38:264-270, 1995.
  3. Avery GB, Villavicencio O, Lilly JR, Randolph JG: Intractable diarrhea in early infancy. Pediatrics, 41:712-722, 1968.
  4. Chong S, Wright V, Nishigame T, et al.: Infantile colitis: a manifestation of intestinal Behcet's syndrome. Journal of pediatric gastroenterology and nutrition, 7:622-627, 1988. https://doi.org/10.1097/00005176-198807000-00025
  5. Davidson G, Cutz E, Hamilton J, Gall D: Familial enteropathy: a syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy. Gastroenterology, 75:783-790, 1978.
  6. Pittock S, Drumm B, Fleming P, et al.: The oral cavity in Crohn's disease. J Pediatr, 138:767-771,2001. https://doi.org/10.1067/mpd.2001.113008
  7. Kasahara Y, Tanaka S, Nishino M, et al.: Intestinal involvement in Behcet's disease: review of 136 surgical cases in the Japanese literature. Diseases of the Colon & Rectum, 24:103-106, 1981. https://doi.org/10.1007/BF02604297
  8. Smith GE, MAJ LRK: The colitis of Behcet's disease: A separate entity? The American journal of digestive diseases, 18:987-1000, 1973. https://doi.org/10.1007/BF01072445
  9. Engelhardt KR, Shah N, Faizura-Yeop I, et al.: Clinical outcome in IL-10- and IL-10 receptor-deficient patients with or without hematopoietic stem cell transplantation. J Allergy Clin Immunol, 131:825-830, 2013. https://doi.org/10.1016/j.jaci.2012.09.025
  10. Shim JO, Hwang S, Yang HR, et al.: Interleukin-10 receptor mutations in children with neonatal-onset Crohn's disease and intractable ulcerating enterocolitis. Eur J Gastroenterol Hepatol, 25:1235-1240,2013.
  11. Rogers RS: Recurrent aphthous stomatitis: clinical characteristics and associated systemic disorders. Seminars in cutaneous medicine and surgery. Elsevier, 278-283, 1997.
  12. Thapar N, Shah N, Ramsay AD, et al.: Long-term outcome of intractable ulcerating enterocolitis of infancy. Journal of pediatric gastroenterology and nutrition, 40:582-588, 2005. https://doi.org/10.1097/01.MPG.0000159622.88342.BC
  13. Thapar N, Lindley KJ, Kiparissi F, et al.:Treatment of intractable ulcerating enterocolitis of infancy by allogeneic bone marrow transplantation. Clinical Gastroenterology and Hepatology, 6:248-250, 2008. https://doi.org/10.1016/j.cgh.2007.11.019
  14. Loftus Jr EV, Tremaine WJ, Habermann TM, et al.: Risk of lymphoma in inflammatory bowel disease. The American journal of gastroenterology, 95:2308-2312, 2000. https://doi.org/10.1111/j.1572-0241.2000.02316.x
  15. Lewis JD, Bilker WB, Brensinger C, et al.: Inflammatory bowel disease is not associated with an increased risk of lymphoma. Gastroenterology,121:1080-1087, 2001. https://doi.org/10.1053/gast.2001.28703
  16. Kim SH, Song JS, Choi HJ, et al.: Early detection of behcet's disease. J Korean Acad Pediatr Dent, 36:575-579, 2009.