Incontinentia pigmenti produces darkly-pigmented swirling marks on the skin. It occurs more frequently in females. The skin lesions are divided into three stages: blisters (vesicles and bullae) are present at birth or within the first 6 to 7 weeks, followed by a rough wart-like (verrucous) stage, and lastly, swirled and bizarre patterns of dark pigmentation (hyperpigmentation) appear.
Review Date 4/14/2017
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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.