Incontinentia Pigmenti: Clinical Observation of 40 Korean Cases.
10.3346/jkms.2006.21.3.474
- Author:
Beom Joon KIM
1
;
Hyo Seung SHIN
;
Chong Hyun WON
;
Jong Hee LEE
;
Kyu Han KIM
;
Myeung Nam KIM
;
Byung In RO
;
Oh Sang KWON
Author Information
1. Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea. oskwon@snu.ac.kr
- Publication Type:Original Article
- Keywords:
Incontinentia Pigmenti;
Skin Diseases, Genetic;
Genodermatosis;
Seizures;
Retinal Diseases
- MeSH:
Stomatognathic Diseases/complications;
Skin Diseases/complications;
Male;
Magnetic Resonance Imaging/methods;
Korea;
Infant, Newborn;
Infant;
Incontinentia Pigmenti/*diagnosis/pathology;
Humans;
Female;
Eye Diseases/complications;
Eosinophilia/complications;
Child, Preschool;
Child;
Central Nervous System Diseases/complications
- From:Journal of Korean Medical Science
2006;21(3):474-477
- CountryRepublic of Korea
- Language:English
-
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, genentic counselors, and even dentists is crucial.
