A Report of Incontinentia Pigmenti in an 11-year-old Girl

 Incontinentia pigmenti (IP) is a rarely diagnosed x-linked dominant disease affecting tissues of ectodermal and mesodermal origin such as cutaneous tissues, teeth, eyes, hair, and the central nervous system. Dermatologic manifestations are often the first signs observed in patients diagnosed with IP and are present in nearly all the subjects, but they are less harmful and do not require treatment. Oral manifestations in patients diagnosed with IP might affect both the deciduous and permanent teeth, with tooth shape anomalies and hypodontia, delayed tooth eruption, cleft palate, and high arched palate. These oral abnormalities influence feeding, quality of life (QoL), and self-esteem of the patient but can be successfully corrected by oral rehabilitation.

 Here, we report the case of a female patient, aged 11 years, presenting with dental manifestations such as hypodontia, conical teeth, delayed tooth eruption, narrow and atrophic dental ridge, and also some non-dental findings of IP. Her dental ‎management included oral hygiene instructions, extraction of all unrestorable primary molars, and composite filling of all primary canines.

 A removable space maintainer was constructed for the patient, which resulted in favorable esthetic outcomes, proper re-establishment of mastication, and improved self-esteem.