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KMID : 0358920090360040563
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Journal of the Korean Academy of Pedodontics
2009 Volume.36 No. 4 p.563 ~ p.567
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INCONTINENTIA PIGMENTI : CASE REPORTS
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Shin Hye-Sung
Kim Seon-Mi
Choi Nam-Ki
Yang Kyu-Ho
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Abstract
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Incontinentia pigmenti(Bloch-Sulzberger syndrome) is a sex-linked hereditary disorder so girls are almost exclusively affected. The frequency rate is approximately 1:40,000 among girls. It is associated with skin, ocular, dental, skeletal and central nervous deformities. We reported 2 cases of medically diagnosed IP patients who were 4 and 5 years old girls. They had thin and sparse hair, and represented congenital missing of multiple primary and permanent teeth, accessory cusps and cone-shaped crowns. Therefore we report the dental manifestations and treatment progress.
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KEYWORD
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Incontinentia pigmenti, Missing tooth, Sex-linkage, Bloch-Sulzberger syndrome
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