Overview
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 6/7/2023
Updated by: Elika Hoss, MD, Assistant Professor of Dermatology, Mayo Clinic, Scottsdale, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.