De novo NEMO gene deletion (delta4-10)--a cause of incontinentia pigmenti in a female infant: a case report.

Incontinentia pigmenti (IP) is a rare, inherited, multisystem genodermatosis. It is transmitted as an X-linked dominant trait. The disorder is a consequence of mutations in the NEMO gene (Xq28) that completely abolish expression of the NF-kappaB essential modulator.