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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.

SUBMITTER: Afshar H

PROVIDER: S-EPMC3484832 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature

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Abnormal dentition in a boy with incontinentia pigmenti: case report.

Afshar H H Daneshpazhooh M M Kiani A A Aref P P Baniameri Z Z

Journal of dentistry (Tehran, Iran) 20120930 3

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 ...[more]

PMID: 23119137

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