Rare Case Report on Incontinentia Pigmenti

Incognentiapigmenti is a neurocutaneous syndrome. It is a x - linked dominant disorder. It is a rare, inheritable, multisystem ectodermal disorder. Pathogenic IKBKG gene or 47, XXY karyotyping seen in female X - inactivation (45, X) seen. Most commonly involved organ is skin. Other systems involved are CNS, ocular, dental and hair.