[Dental anomalies associated with incontinentia pigmenti or Bloch-Sulzberger syndrome]

Abstract

We report dental anomalies related to the Bloch- Sulzberger Syndrome better known as Incontinentia Pigmenti (IP). IP is an X related hereditary disease that occurs for about 95% of all patients in females and is lethal for the male foetus. Because of severe skin lesions in the neonatal period the disease is more known by dermatologists than dentists. Beside the skin lesions other associated deficiencies can be present like: ocular injuries, anomalies of hair and nails, defects of the central nervous system, malformations of the extremities and anomalies of the teeth. We report 8 female patients of different ages, out of 5 families. All were diagnosed with IP after DNA examination. Dental manifestations related to IP are: delayed eruption, oligodontia, agenesis, peg-shaped or malformed teeth, supernumerary teeth and supplementary cusps. IP is under diagnosed if the symptoms are limited to agenesis of only a few teeth and inconspicuous skin lesions. Dentists are ideally placed to identify individuals with IP and should refer patients with a presumption of IP to proper medical specialists.