CLINICAL REPORT OF NEUROFIBROMATOSIS TYPE 1 PATIENT

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Journal of the Korean Academy of Pediatric Dentistry 2011;38(2):187-193.
Published online May 31, 2011.

CLINICAL REPORT OF NEUROFIBROMATOSIS TYPE 1 PATIENT

전북대학교 치과대학 소아치과학교실 및 구강생체과학연구소

제1형 신경섬유종증 환아의 임상적 치험례

이대우, 양연미, 김재곤, 백병주, 소유려

전북대학교 치과대학 소아치과학교실 및 구강생체과학연구소

Correspondence: ,

Received: 10 December 2010 • Accepted: 21 March 2011

Abstract

Neurofibromatosis is known as an autosomal dominant disorder caused by a mutation of a tumor suppressor gene on the long arm of chromosome 17 that affects the bone, nervous system, soft tissue, skin, and endocrine system. The most characteristic finding, which is helpful for clinical diagnosis as well, is the neurofibroma. Likewise brown macules called Cafe-au-lait spots with color of caffee latte, and Lisch nodules found around iris are useful to diagnose the disease. As known, the possibility of the neurofibromatosis occurred in oral cavity is relatively rare, and in most of cases it is related to soft tissue changes with single or multiple neurofibromatosis. The purpose of this report is to present characteristic dental findings which were found in a 4-year-old male and his father, both diagnosed as neurofibromatosis at Chonbuk National University Hospital before visiting our department, pediatric dentistry.

Key Words: Neurofibromatosis, Neurofibroma, Cafe-au-lait spot, Lisch nodules