INCONTINENTIA PIGMENTI : CASE REPORTS

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Journal of the Korean Academy of Pediatric Dentistry 2009;36(4):563-567.
Published online November 30, 2009.

INCONTINENTIA PIGMENTI : CASE REPORTS

전남대학교 치의학전문대학원 소아치과학교실, 치의학연구소 및 2단계 BK21

색소실조증 환아의 치험례

신혜성, 김선미, 최남기, 양규호

전남대학교 치의학전문대학원 소아치과학교실, 치의학연구소 및 2단계 BK21

Correspondence: ,

Received: 13 May 2009 • Accepted: 14 August 2009

Abstract

ncontinentia 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.

Key Words: Incontinentia pigmenti; Missing tooth; Sex-linkage; Bloch-Sulzberger syndrome