색소실조증 환자의 치과적 특성 : 증례보고

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.