Incontinentia Pigmenti
Abstract
Incontinentia pigmenti is an X-linked dominant disorder with characteristic skin lesions and anomalies of teeth, hair, nails, eyes, and central nervous system. Cutaneous lesions are the most common identifiable abnormality and characterized in 4 stages. ONS abnormalities are the cause of most morbidity in this disorder. The NEMO gene is identified with this disorder. In this update, Drs. David Hsieh and Bhagwan Moorjani from Children's National Medical Center in Washington, DC highlight the reporting of reversible brain lesions by MRI, and the addition of the gothic palate in the spectrum of oral and dental anomalies in incontinentia pigmenti.
Document Details
- Document Type
- Technical Report
- Publication Date
- Jan 01, 2008
- Accession Number
- ADA478060
Entities
People
- Bhagwan Moorjani
- David T. Hsieh