Incontinentia pigmenti: clinical observation of 40 Korean cases

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Kim, Beom Joon; Shin, Hyo Seung; Won, Chong Hyun; Lee, Jong Hee; Kim, Kyu Han; Kim, Myeung Nam; Ro, Byung In; Kwon, Oh Sang
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Korean Academy of Medical Science
J Korean Med Sci. 2006 Jun;21(3):474-7.
Central Nervous System Diseases/complicationsChildChild, PreschoolEosinophilia/complicationsEye Diseases/complicationsFemaleHumansIncontinentia Pigmenti/*diagnosis/pathologyInfantInfant, NewbornKoreaMagnetic Resonance Imaging/methodsMaleSkin Diseases/complicationsStomatognathic Diseases/complications
Incontinentia pigmenti (IP) is an uncommon genodermatosis that usually occurs in female infants. It is characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The aim of this study was to clarify clinical symptoms, accompanying diseases, and complications of IP. Forty cases of IP have been reviewed by their medical records, laboratory data, clinical photographs, and telephone survey. Male-to-female ratio was 1 to 19 and their onsets were mostly in utero. They were usually diagnosed during the neonatal period owing to their early expression of skin manifestation. Central nervous system anomalies were found in 46.7%. Ocular disorders and dental defects were detected in 66.7% and 72.7% respectively. The most commonly diagnosed anomalies were hypodontia, retinopathy, and seizure. For better understanding of IP, long term and close cooperation between dermatologists, pediatricians, neuroscientists, genetic counselors, and even dentists is crucial.
1011-8934 (Print)
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College of Medicine/School of Medicine (의과대학/대학원)Dermatology (피부과학전공)Journal Papers (저널논문_피부과학전공)
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