title: Phacomatosis pigmentokeratotica without extracutaneous abnormalities: a case study involving a preterm baby.
abstract: 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.
authors.affiliation: Department of Dermatology, Korea University College of Medicine, Seoul, Korea.
authors.forename: Ga Na
authors.initials: GN
authors.lastname: Oh
authors.forename: Jong Yeob
authors.initials: JY
authors.lastname: Kim
authors.forename: Jae Eun
authors.initials: JE
authors.lastname: Choi
authors.forename: Hyo Hyun
authors.initials: HH
authors.lastname: Ahn
authors.forename: Young Chul
authors.initials: YC
authors.lastname: Kye
authors.forename: Soo Hong
authors.initials: SH
authors.lastname: Seo
mesh_terms: D003937:Diagnosis, Differential; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D008297:Male; D009508:Nevus, Pigmented / Q000175:diagnosis* / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000473:pathology