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http://dx.doi.org/10.5933/JKAPD.2015.42.2.180

Incontinentia Pigmenti with Multiple Missing Teeth : Case Reports  

Choi, Shinae (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University)
Kim, Youngjin (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University)
Nam, Soonhyeun (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University)
Kim, Hyunjung (Department of Pediatric Dentistry,School of Dentistry, Kyungpook National University)
Publication Information
Journal of the korean academy of Pediatric Dentistry / v.42, no.2, 2015 , pp. 180-187 More about this Journal
Abstract
Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.
Keywords
Incontinentia pigmenti; Congenital missing; Ectodermal dysplasia;
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Times Cited By KSCI : 1  (Citation Analysis)
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