난치성 궤양성 소장결장염 영아의 구강 내 증상과 감별진단: 증례보고
Oral Symptoms of Intractable Ulcerating Enterocolitis of Infancy and Differential Diagnosis: A Case Report
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난치성 궤양성 소장결장염은 영아에게서 드물게 나타나는 만성 염증성 장 질환으로 1991년 처음으로 보고되었다. 난치성궤양성 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 결장절제술을신속하게 시행함으로써 증상의 완화와 질병의 진행을 막는데 효과적으로 대처할 수 있으므로 매우 중요하다. 또한 대부분의난치성 궤양성 소장결장염 환아에게서 구강 내 궤양이 가장 먼저 발현되므로 이 질환의 증상과 특징에 대해 아는 것은 매우의미 있을 것으로 사료되어 보고하는 바이다.
Abstract
Intractable ulcerating enterocolitis is an uncommon inflammatory bowel disease syndrome of neonatal onsetfirst described in 1991. Intractable ulcerating enterocolitis usually presents in the neonate with a mouth ulcerationand 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 differentialdiagnosis 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 hospital3weeks later with blood diarrhea. As a result of endoscopy, there were large ulcerating lesions and the patientwas diagnosed intractable ulcerating enterocolitis. Early recognition of Intractable ulcerating enterocolitis appears to be beneficial because colectomy, as opposedto immunosuppression, appears to be effective in controlling disease symptoms and progression. Most of theinfants who were affected intractable ulcerating enterocolitis were normal at birth and oral manifestationappeared earlier than others. So, it is very meaningful for dentists to know about Intractable ulcerating enterocolitis.
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