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Incontinentia pigmenti presenting as hypodontia in a 3-year-old girl: a case report.
Kitakawa, Dárcio; Fontes, Patrícia Campos; Magalhães, Fernando Augusto Cintra; Almeida, Janete Dias; Cabral, Luiz Antonio Guimarães.
Afiliação
  • Kitakawa D; Department of Bioscience and Oral Diagnosis, São José dos Campos Dental School, São Paulo State University-UNESP, São José dos Campos-SP, Brazil.
J Med Case Rep ; 3: 116, 2009 Nov 10.
Article em En | MEDLINE | ID: mdl-19946534
INTRODUCTION: 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. CASE PRESENTATION: 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. CONCLUSION: Hypodontia is a frequent finding in incontinentia pigmenti, and dentists should be aware of this condition in order to help with the diagnosis.

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: J Med Case Rep Ano de publicação: 2009 Tipo de documento: Article País de afiliação: Brasil País de publicação: Reino Unido

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: J Med Case Rep Ano de publicação: 2009 Tipo de documento: Article País de afiliação: Brasil País de publicação: Reino Unido