Home > Encyclopedia of Neurological Disorders > Incontinentia Pigmenti

Definition

Incontinentia pigmenti is a rare genetic disease resulting in a neurocutaneous disorder. Neurocutaneous means that the disorder affects the nervous system and that clinical abnormalities can involve the skin, hair, and teeth of affected individuals.

Description

Incontinentia pigmenti patients develop discolored, abnormally pigmented skin that is distributed randomly and asymmetrically. Occasionally, persons with incontinentia pigmenti experience cognitive delays (including mental retardation), but most have normal intelligence. Muscle weakness in one or both sides of the body is also characteristic of the disorder. Incontinentia pigmenti is also known as Bloch-Sulzberger syndrome, as well as incontinentia pigmenti, type 2.

Demographics

Incontinentia pigmenti is considered rare, with only about...