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J Korean Med Sci. 2006 Jun;21(3):474-7.

Incontinentia pigmenti: clinical observation of 40 Korean cases.

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1
Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea.

Abstract

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.

PMID:
16778392
PMCID:
PMC2729954
DOI:
10.3346/jkms.2006.21.3.474
[Indexed for MEDLINE]
Free PMC Article
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