Incontinentia pigmenti causes swirling pigmentation of the skin. It is seen more frequently in females. The skin lesions are divided into three stages. Blistering (with vesicles and bullae) present at birth or within 6 to 7 weeks, a verrucous (rough wart-like stage), and the final stage shows whorled and bizarre patterns of dark pigmentation (hyperpigmentation).
Review Date 4/14/2015
Updated by: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.