대한소아치과학회지 (Journal of the korean academy of Pediatric Dentistry)

  • 제36권4호
  • /
  • Pages.563-567
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  • 2009
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  • 1226-8496(pISSN)
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  • 2288-3819(eISSN)
대한소아치과학회 (Korean Academy of Pediatric Dentistry)
권호 표지

색소실조증 환아의 치험례

INCONTINENTIA PIGMENTI : CASE REPORTS

  • 신혜성 (전남대학교 치의학전문대학원 소아치과학교실, 치의학연구소 및 2단계 BK21) ;
  • 김선미 (전남대학교 치의학전문대학원 소아치과학교실, 치의학연구소 및 2단계 BK21) ;
  • 최남기 (전남대학교 치의학전문대학원 소아치과학교실, 치의학연구소 및 2단계 BK21) ;
  • 양규호 (전남대학교 치의학전문대학원 소아치과학교실, 치의학연구소 및 2단계 BK21)
  • Shin, Hye-Sung (Department of pediatric dentistry, Chonnam national university school of dentistry, Dental Research Institute and Second stage of BK21) ;
  • Kim, Seon-Mi (Department of pediatric dentistry, Chonnam national university school of dentistry, Dental Research Institute and Second stage of BK21) ;
  • Choi, Nam-Ki (Department of pediatric dentistry, Chonnam national university school of dentistry, Dental Research Institute and Second stage of BK21) ;
  • Yang, Kyu-Ho (Department of pediatric dentistry, Chonnam national university school of dentistry, Dental Research Institute and Second stage of BK21)
  • 발행 : 2009.11.30
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초록

색소실조증(Incontinentia pigmenti)은 반성우성으로 유전되며, 대부분 여아에서 발생하고 여아 4만 명 중 한 명에서 발생하는 드문 질환이다. 증상으로는 색소침착, 수포형성, 안구 증상, 치아 이상, 골격계 이상, 신경계 이상 등을 동반할 수 있다. 본 증례는 의과적으로 색소실조증으로 진단받고, 치아 결손을 주소로 내원한 5세 여아와, 4세 여아이다. 이들은 가늘고 성긴 머리카락을 나타내었으며, 구강 내 증상으로는 다수 유치와 영구치의 결손, 과잉교두, 원뿔형 치관형태 등을 나타내었다. 이에 구강 증상 및 치료 경과를 보고하는 바이다.

Incontinentia pigmenti(Bloch-Sulzberger syndrome) is a sex-linked hereditary disorder so girls are almost exclusively affected. The frequency rate is approximately 1:40,000 among girls. It is associated with skin, ocular, dental, skeletal and central nervous deformities. We reported 2 cases of medically diagnosed IP patients who were 4 and 5 years old girls. They had thin and sparse hair, and represented congenital missing of multiple primary and permanent teeth, accessory cusps and cone-shaped crowns. Therefore we report the dental manifestations and treatment progress.

키워드

참고문헌

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