Incontinentia pigmenti: three cases with unusual features.

Abstract

Three patients with incontinentia pigmenti are described who illustrate some of the unusual features of this uncommon genodermatosis. One child with skin, ophthalmologic, and dental findings had atrophic, hypopigmented streaks on her legs by the age of 2 1/2 years that were consistent with the fourth stage of incontinentia pigmenti. This child's mother, who also had incontinentia pigmenti, had identical atrophic streaks on the legs, as well as irregular axillary pigmentation, scarring alopecia, and dental abnormalities. A second child is described who had annular blisters, persistent verrucous plaques, whorled hyperpigmentation, and dental abnormalities.