Incontinentia Pigmenti

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 Childrens 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.