Incontinentia Pigmenti: Clinical Observation of 40 Korean Cases
Journal of Korean Medical Science
;
: 474-477, 2006.
Article
in English
| WPRIM
| ID: wpr-47130
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Skin Diseases
/
Incontinentia Pigmenti
/
Magnetic Resonance Imaging
/
Stomatognathic Diseases
/
Central Nervous System Diseases
/
Eosinophilia
/
Eye Diseases
/
Korea
Limits:
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
/
Infant, Newborn
Country/Region as subject:
Asia
Language:
English
Journal:
Journal of Korean Medical Science
Year:
2006
Type:
Article
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