title: Incontinentia pigmenti presenting as hypodontia in a 3-year-old girl: a case report.
abstract: BACKGROUND
  Incontinentia pigmenti or Bloch-Sulzberger syndrome is a rare X-linked dominant disease that mainly affects the skin, eyes, hair, central nervous system and teeth. The disease is predominant among women. Although dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome, they are less damaging to the patient and do not require treatment. However, oral involvement characterized by hypodontia of deciduous and permanent teeth is important for the diagnosis and treatment of the patient.
  
  
  METHODS
  We report the case of a 3-year-old girl with ophthalmologic and neurologic disturbances, cutaneous manifestations and hypodontia. Since the patient did not present more damaging manifestations such as neurologic and/or ophthalmologic problems, her most severe complications were related to dental anomalies. The importance of integrated dental treatment, which combines pediatric dentistry, orthodontics and conventional prosthesis, is emphasized.
  
  
  CONCLUSIONS
  Hypodontia is a frequent finding in incontinentia pigmenti, and dentists should be aware of this condition in order to help with the diagnosis.
authors: Department of Bioscience and Oral Diagnosis, São José dos Campos Dental School, São Paulo State University-UNESP, São José dos Campos-SP, Brazil.
authors: Dárcio
authors: D
authors: Kitakawa
authors: Patrícia Campos
authors: PC
authors: Fontes
authors: Fernando Augusto Cintra
authors: FA
authors: Magalhães
authors: Janete Dias
authors: JD
authors: Almeida
authors: Luiz Antonio Guimarães
authors: LA
authors: Cabral