J Korean Acad Pediatr Dent 41(1) 2014 ISSN (print) ISSN (online) Oral Symptoms of Int

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1 ISSN (print) ISSN (online) Oral Symptoms of Intractable Ulcerating Enterocolitis of Infancy and Differential Diagnosis: A Case Report Hyoseon Min, Hyungjun Choi, Jaeho Lee, Byungjai Choi, Hyoseol Lee Department of Pediatric Dentistry, College of Dentistry, Yonsei University Abstract Intractable ulcerating enterocolitis is an uncommon inflammatory bowel disease syndrome of neonatal onset first described in 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. Key words : Intractable ulcerating enterocolitis, Aphthous ulcer, Behcet s disease, Crohn s disease Ⅰ. 서론특발성염증성장질환 (Idiopathic inflammatory bowel disease, IBD) 은영아에서는매우드문질환으로이중난치성궤양성소장결장염 (intractable ulcerating enterocolitis, IE) 는신생아에서의발생이 1991년 Sanderson 등 1) 에의해처음으로보고되었다. 출생시는정상이나생후약 2개월경부터혈변또는농액성설사증세를보이기시작하며구강내궤양, 대장의플라스크모양의큰궤양, 소장의조직생검상미세융모의부분적퇴화등이특징적소견이다 2). 빠른발현시기와높은가족성발병양상등으로인해상염색체열성질환으로여겨지며크론병및베체트병과의감별진단을요한다 3-5). 크론병은영아에서는매우드물고 IE가주로대장을침범하는데비해주로소장을침범하며간혹대장에서도병변이발견될수있으며조직병리학적소견을통해서도크론병과 IE를감별할수있다 5,6). 베체트병은대장을침범할경우본질환과임상양상이매우유사하다. 하지만베체트병의주요진단기준인성기부위궤양, 아프타성구내염, 눈의염증, 피부병변중구강내궤양을제외한임상증상은발견되지않으며조직소견에서도베체트병에서특징적인혈관염을볼수없다 7,8). 저자는구강내궤양이있어이와관련된전신질환을감별하기위해본원소아치과에내원한환아를계기로 IE에대한다소의지견을얻었기에보고하는바이다. Corresponding author : Hyoseol Lee Department of Pediatric Dentistry, College of Dentistry, Yonsei University, 50 Yonsei-ro, Seodaemun-gu, Seoul, , Korea Tel: / Fax: / specialdentist@yuhs.ac Received September 2, 2013 / Revised December 10, 2013 / Accepted December 10,

Ⅱ. 증례보고생후 2주경부터시작된구강내궤양으로본원소아청소년과에입원중이던생후 18일된환아가구강내궤양으로감별할수있는전신질환에대해알아보고자본과로의뢰되었다. 출생당시몸무게 3.3 kg으로별다른특이사항없었으며본과내원당시에도구강내궤양외에뚜렷한증상없었으나, 환아의형역시구강내궤양이선행된원인불명의장염으로생후 50일경사망한가족력이있었다.

이비인후과평가결과구강뿐아니라구강에서하인두부에이르기까지궤양이널리퍼져있음이확인되었으며 (Fig. 2), 그후대장내시경, 면역학적검사, 유전자검사, 미생물, 혈액, 화학검사상에도별다른원인을발견하지못하고퇴원하였다. 환아는그로부터 3주뒤혈변과고열이나타나면서재입원하였다.

이질환을 1990년처음으로명명한 Sanderson 등 1) 은영아기 IE를크론병이나베체트병과독립된질환으로분류하였으며이들의보고에따르면증례보고된 5명모두에서생후 2개월이내에혈변또는점액성의설사를보였다. 특히 5명모두에서구강내궤양, 4명에서치루를관찰할수있었으며모든임상증상중구강내아프타성궤양이가장선행되어나타났다 1).

2 Ⅱ. 증례보고생후 2주경부터시작된구강내궤양으로본원소아청소년과에입원중이던생후 18일된환아가구강내궤양으로감별할수있는전신질환에대해알아보고자본과로의뢰되었다. 출생당시몸무게 3.3 kg으로별다른특이사항없었으며본과내원당시에도구강내궤양외에뚜렷한증상없었으나, 환아의형역시구강내궤양이선행된원인불명의장염으로생후 50일경사망한가족력이있었다. 본과내원시환아는섭식곤란으로인해전신적으로쇠약한모습을보였으며구개부후방에서불규칙한경계를가진약 5~7 mm 크기의두개의아프타성궤양이관찰되었다 (Fig. 1). 베체트병을염두에두고환자의사지및생식기계를시진한결과별다른이상을발견할수없었으나증상이아직발현되지않았을가능성이있으므로추가적인면역학적검사를시행해볼것과구강칸디다증에대한진균도말검사가필요할수있음을설명하였다. 이비인후과평가결과구강뿐아니라구강에서하인두부에이르기까지궤양이널리퍼져있음이확인되었으며 (Fig. 2), 그후대장내시경, 면역학적검사, 유전자검사, 미생물, 혈액, 화학검사상에도별다른원인을발견하지못하고퇴원하였다. 환아는그로부터 3주뒤혈변과고열이나타나면서재입원하였다. 대장내시경에서대장내거대궤양이관찰되었으며혈변과농액성설사가지속되어면역억제제를투약하였으나별다른차도를보이지않았으며결장전절제술을시행하였다 (Fig. 3). 대장내시경및조직검사등을토대로 IE로진단되었으며현재까지악화와호전을반복하며본원소아청소년과에내원중이다. Ⅲ. 총괄및고찰 IE은매우드문질환으로대다수에서구강내아프타성궤양이선행되는유전적인만성염증성장질환이다 2). 이질환을 1990년처음으로명명한 Sanderson 등 1) 은영아기 IE를크론병이나베체트병과독립된질환으로분류하였으며이들의보고에따르면증례보고된 5명모두에서생후 2개월이내에혈변또는점액성의설사를보였다. 특히 5명모두에서구강내궤양, 4명에서치루를관찰할수있었으며모든임상증상중구강내아프타성궤양이가장선행되어나타났다 1). 또한보고된증례에서자가면역항체에대한검사소견상평활근, 위벽세포, 갑상선및장세포에대한자가면역항체는모두음성이었으며특징적으로모든환아에서 IgA 증가를보였다 1,3). 병인은확실하게밝혀지지않았으나장내세균총에대한면역학적이상으로초래된질환으로여겨지며최근영아기 IBD 가 interleukin-10 (IL-10) 및 interleukin-10 receptor (IL- 10R) 결함과관계가있다는연구에이어한국에서도영아기에크론병및 IE로진단된환자들에서 IL-10RA에결함이있음이보고되었다 9,10). 구강내궤양은베체트병에서나타나는것과매우유사한 complex aphthosis 소견을특징적으로보이며이러한형태의구강궤양을동반할수있는전신질환들과의감별진단이필요 Fig. 1. Oral cavity showing two aphthous ulcers in the posterior palatal area. Fig. 2. Ulceration extended not only oral cavity but also epiglottis and hypopharynx. Fig. 3. Perianal view : Daily dressing due to perianal abscess. 81

3 Table 1. Aphthous ulcer associated systemic disease Ulcus vulvae acutum Behcet's disease Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome Fever, aphthosis, pharyngitis, and adenitis (FAPA) syndrome Cyclic neutropenia Aphthous-like ulceration of HIV disease Hematinic deficiencies Celiac disease(sprue, gluten-sensitive enteropathy) Inflammatory bowel disease 하다 (Table 1) 11). Nikhil Thapar 등 12) 에따르면 IE는가족성발현, 신생아시기에발병, 뚜렷하게나타나는구강과항문주위의궤양및염증, 소장근위부의부분적인미세융모의단축과장샘이길어지는양상, 점막하층까지펼쳐져있는플라스크모양의깊은궤양성병변, 면역억제치료에반응을보이지않는특징을갖는다. 따라서현재까지는조기에결장전절제술을시행하는것이가장효과적인치료법이며최근에는동종골수이식법을통한치료사례가소개되기도하였다 13). 특히장기적인고농도의면역억제제의사용이향후림프종위험을높일수있다는보고가있어조기에정확한진단을내리는것이중요하다 14,15). 보고된증례에서임상경과를보면모든환자가결장절제술을받았으며면역억제요법은효과가없었으며생존한경우재발하는구강궤양과치루가문제가되었다. 재발하는구강궤양에대해서는베체트병환자에게서나타난구강궤양과동일하게연고, 양치액, 젤또는도포용 corticosteroid를사용하여염증을조절하고섭식시동통을해소하기위해국소도포용 Lidocaine을함께처방할수있을것으로보인다 16). Ⅳ. 요약 구강궤양과연관된전신질환으로는일반적으로크론병이나베체트병을생각해볼수있다. 하지만이두질환은신생아에서는거의발병되지않는것으로알려져있으며조직학적, 임상적특징이 IE와다르다. IE는전위장관에걸친 ulcer 와혈변, 조직학적소견상 flask shape의 ulceration 을보이는질환으로정확한원인은알려져있지않으나상염색체열성유전을하는면역질환의한종류로생각된다. 매우드문질병이지만구강내궤양이동반되고크론병이나베체트병과마찬가지로전신증상이발현하기전에구강증상이나타나므로신생아에서구강내궤양이발견될경우의과적평가와소아과와의협진이반드시필요할것으로생각된다. References 1. Sanderson I, Risdon R, Walker-Smith J: Intractable ulcerating enterocolitis of infancy. Archives of disease in childhood, 66: , Jeong JY, Seo JK, KW P, Chi JG: A Case of Intractable Ulcerating Enterocolitis of Infants. Korean journal of Pediatrics, 38: , Avery GB, Villavicencio O, Lilly JR, Randolph JG: Intractable diarrhea in early infancy. Pediatrics, 41: , Chong S, Wright V, Nishigame T, et al.: Infantile colitis: a manifestation of intestinal Behcet s syndrome. Journal of pediatric gastroenterology and nutrition, 7: , 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: , Pittock S, Drumm B, Fleming P, et al.: The oral cavity in Crohn s disease. J Pediatr, 138: , 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: , Smith GE, MAJ LRK: The colitis of Behcet s disease: A separate entity? The American journal of digestive diseases, 18: , 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: , 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: , Rogers RS: Recurrent aphthous stomatitis: clinical characteristics and associated systemic disorders. Seminars in cutaneous medicine and surgery. Elsevier, , Thapar N, Shah N, Ramsay AD, et al.: Long-term outcome of intractable ulcerating enterocolitis of infancy. Journal of pediatric gastroenterology and nutrition, 40: , Thapar N, Lindley KJ, Kiparissi F, et al.: 82

4 Treatment of intractable ulcerating enterocolitis of infancy by allogeneic bone marrow transplantation. Clinical Gastroenterology and Hepatology, 6: , Loftus Jr EV, Tremaine WJ, Habermann TM, et al.: Risk of lymphoma in inflammatory bowel disease. The American journal of gastroenterology, 95: , Lewis JD, Bilker WB, Brensinger C, et al.: Inflammatory bowel disease is not associated with an increased risk of lymphoma. Gastroenterology, 121: , Kim SH, Song JS, Choi HJ, et al.: Early detection of behcet s disease. J Korean Acad Pediatr Dent, 36: ,

5 국문초록 난치성궤양성소장결장염영아의구강내증상과감별진단 : 증례보고 민효선 최형준 이제호 최병재 이효설 연세대학교치과대학소아치과학교실 난치성궤양성소장결장염은영아에게서드물게나타나는만성염증성장질환으로 1991년처음으로보고되었다. 난치성궤양성소장결장염은주로영아기에구강내궤양에서시작되어항문주위및장질환으로진행되는특징을갖는다. 생후 18일된환아가구강내궤양으로감별할수있는전신질환에대해알아보고자본과로의뢰되었다. 당시구강내궤양을제외한특이사항은없었으며 3주뒤혈변이발생하였다. 대장내시경상에서대장내거대궤양이발견되었으며여러검사결과난치성궤양성소장결장염으로진단되었다. 치과의사가난치성궤양성소장결장염을조기에진단하는것은면역억제제에반응이없는본질환에대해결장절제술을신속하게시행함으로써증상의완화와질병의진행을막는데효과적으로대처할수있으므로매우중요하다. 또한대부분의난치성궤양성소장결장염환아에게서구강내궤양이가장먼저발현되므로이질환의증상과특징에대해아는것은매우의미있을것으로사료되어보고하는바이다. 주요어 : 난치성궤양성소장결장염, 아프타성구내염, 베체트병, 크론병 84

Lumbar spine

Lumbar spine CT 32 111 DOI : 10.3831/KPI.2010.13.2.111 Lumbar Spine CT 32 Received : 10. 05. 23 Revised : 10. 06. 04 Accepted : 10. 06. 11 Key Words: Disc herniation, CT scan, Clinical analysis The Clinical