incontinentia pigmenti /inkon′tinen′shə pigmen′tī/ , an X-linked dominant syndrome with onset at birth or shortly thereafter that is usually lethal in males. It is characterized by the presence of brown or slate-brown bands, whorls, swirls, or splatter-like hyperpigmented cutaneous lesions that are preceded by vesiculobullous and verrucous inflammatory changes and are often associated with developmental anomalies involving other structures, such as the hair, eyes, and skeletal and central nervous systems. Also called Bloch-Sulzberger incontinentia pigmenti, Bloch-Sulzberger syndrome.
