Dental defects in incontinentia pigmenti: case report.

Incontinentia pigmenti is an uncommon type of ectodermal dyspIasia involving abnormalities of the skin, hair, central nervous system, and teeth. The literature is reviewed and a case with a variety of dental abnormalities is presented. Incontinentia pigrnenti (IP) is one of the lesser known ectodermal dysplasias with fewer than 800 cases reported in the world literature. This genodermatosis affects mostly females and may involve the skin, hair, nails, eyes, and central nervous system. Diagnosis usually is made from the clinical presentation and occasionally skin biopsy. Characteristic changes in the dentition of some patients also have been observed. Dental abnormalities include hypodontia, pegged incisors and canines, and delayed eruption (Butterworth and Ladda 1981; Freira-Maia and Pinheiro 1984). The authors report a case of a 12-year-old female with incontinentia pigmenti associated with enamel hypocalcification and hypoplasia of the permanent teeth.