Incontinentia Pigmenti; a Rare Multisystem Disorder: Case Report of a 10-Year-Old Girl

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. additionally, other ectodermal tissues may be affected such as the central nervous system, eyes, hair, nails and teeth. the disease has an x-linked dominant inheritance pattern. but in our case, there was a mutation in the body cells due to incontinentia pigmenti. the dermatological findings occur in four successive phases. we report the case of a 10-year-old female presented cutaneous, dental and ophthalmic characteristic with 3 years follow-up. dental anomalies such as hypodontia, peg-shaped anterior teeth, malformed primary and permanent teeth, and delayed eruption were seen in our patient.