The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL CHARACTERISTICS OF A PATIENT WITH INCONTINENTIA PIGMENTI : A CASE REPORT

색소실조증 환자의 치과적 특성 : 증례보고

  • Song, Jihyeo (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Koeun (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Song, Je Seon (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Jaeho (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Choi, Hyung-Jun (Department of Pediatric Dentistry, College of Dentistry, Yonsei University)
  • 송지혜 (연세대학교 치과대학 소아치과학교실) ;
  • 이고은 (연세대학교 치과대학 소아치과학교실) ;
  • 송제선 (연세대학교 치과대학 소아치과학교실) ;
  • 이제호 (연세대학교 치과대학 소아치과학교실) ;
  • 최형준 (연세대학교 치과대학 소아치과학교실)
  • Received : 2018.07.12
  • Accepted : 2018.08.06
  • Published : 2018.12.31
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Abstract

Incontinentia pigmenti(IP), or Block-Sulzberger syndrome is a rare X-linked dominant genodermatosis that affects almost in female infant and is usually lethal for males in utero. IP is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. Dental problems are congenital missing of teeth, delayed eruption, abnormal crown shape. We reported a case of 5 year-old female with IP. She had congenital missing of multiple primary and permanent teeth, accessory cusp and cone-shaped crowns. Systemically, she had a problem of retina and hyperpigmented macules on her trunk and extremities as typical character of IP. As the syndrome involves many different medical problems and needs comprehensive consideration. Dental care should be performed in a multidisciplinary consultation system.

본 증례는 유전적 피부질환의 하나로 피부의 착색과 망막의 이상 그리고 다수의 유치 및 영구치 결손과 치아의 형태 이상을 나타내는 색소실조증 환자에 대한 보고이다. 현재 환자는 큰 불편감 없이 유치를 유지하고 있으며 향후 영구치열기까지 정기적인 관찰과 관리가 필요하며, 영구치열 완성 후 영구적인 보철적 수복이 필요할 것으로 사료된다.

Keywords

KDOHJY_2018_v14n2_88_f0001.png 이미지

Fig. 1. Intraoral view.

KDOHJY_2018_v14n2_88_f0002.png 이미지

Fig. 2. Panoramic radiograph : multiple primary and permanent teeth were absent.

KDOHJY_2018_v14n2_88_f0003.png 이미지

Fig. 3. Hyperpigmented lesions are seen on arm and left face.

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