색소실조증 환자의 치과적 특성 : 증례보고
Dental Characteristics of a Patient with Incontinentia Pigmenti : A Case Report
송지혜, 이고은, 송제선, 이제호, 최형준,
소속 상세정보
송지혜 ( Song Ji-Hyeo ) - 연세대학교 치과대학 소아치과학교실
이고은 ( Lee Ko-Eun ) - 연세대학교 치과대학 소아치과학교실
송제선 ( Song Je-Seon ) - 연세대학교 치과대학 소아치과학교실
이제호 ( Lee Jae-Ho ) - 연세대학교 치과대학 소아치과학교실
최형준 ( Choi Hyung-Jun ) - 연세대학교 치과대학 소아치과학교실
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.
키워드
Incontinentia pigmenti; Bloch-Sulzberger syndrome; Congenital missing
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