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Abnormal dentition in a boy with incontinentia pigmenti: case report
H Afshar1, M Daneshpazhooh, A Kiani
1Associate Professor, Dental Research Center and 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.
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