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KMID : 0191120120270111444
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Journal of Korean Medical Science
2012 Volume.27 No. 11 p.1444 ~ p.1446
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Phacomatosis Pigmentokeratotica without Extracutaneous Abnormalities: A Case Study Involving a Preterm Baby
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Oh Ga-Na
Kim Jong-Yeob
Choi Jae-Eun
Ahn Hyo-Hyun
Kye Young-Chul
Seo Soo-Hong
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Abstract
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Phacomatosis pigmentokeratotica (PPK) is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. A preterm male infant of only 830 g at 27 week gestational age had an organoid nevus showing sebaceous differentiation. Also, he had multiple speckled-lentiginous nevus. Correlating the observed clinical presentation with the histopathological findings, the diagnosis of PPK was established. There have been less than 10 cases of PPK without extracutaneous manifestation. We present an uncommon case of a preterm patient with PPK who had no extracutaneous abnormalities.
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KEYWORD
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Epidermal Nevus Syndromes, Phacomatosis Pigmentokeratotica, Preterm Baby
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