Author/Authors :
Afshar, H Associate Professor - Dental Research Center and Department of Pediatric Dentistry - Tehran University of Medical Sciences, Tehran, Iran , Daneshpazhooh, M Associate Professor - Department of Dermatology - Tehran University of Medical Sciences, Tehran, Iran , Kian, A Postgraduate Student - Department of Dermatology - Tehran University of Medical Sciences, Tehran, Iran , Aref, i P Pedodontist - Tehran, Iran , Baniamer, Z Postgraduate Student - Department - of Pediatric Dentistry - Tehran University of Medical Sciences, Tehran, Iran
Abstract :
Incontinentia pigmenti (IP) is an X-linked dominant genodermatosis characterized by typical skin lesions along Blaschko's lines and associated with ocular, dental, nails, hair, skeletal, central nervous system and cardiovascular anomalies. We report a 5-year-old boy with cutaneous hyperpigmentation along Blaschko's lines, atrophic streaks, strabismus and mental retardation. He showed the characteristic abnormal dentition seen in IP as partial hypodontia, peg-shaped anterior teeth and un-erupted teeth. The expression of IP in boys is exceptional as the disease is lethal in males.
