title: Abnormal dentition in a boy with incontinentia pigmenti: case report.
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.
authors.affiliation: Associate Professor, Dental Research Center and Department of Pediatric Dentistry, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: H
authors.initials: H
authors.lastname: Afshar
authors.forename: M
authors.initials: M
authors.lastname: Daneshpazhooh
authors.forename: A
authors.initials: A
authors.lastname: Kiani
authors.forename: P
authors.initials: P
authors.lastname: Aref
authors.forename: Z
authors.initials: Z
authors.lastname: Baniameri
keywords: Dentition; Incontinentia Pigment; Male