Incontinentia pigmenti (IP) is a rare X-linked dominant disease that affects the ectodermal tissues. It is associated with a whorled pattern of scarring alopecia, which is possibly underreported. This scarring alopecia could be used as a marker to identify adult women affected with IP as older patients may have minimal cutaneous manifestations. We report an unusual case of an 8-year-old girl who had recurrent inflammatory stage IP and later developed whorled scarring alopecia in the vertex of the scalp.
Keywords: Incontinentia pigmenti; scarring alopecia; whorled alopecia.