Incontinentia pigmenti presenting as hypodontia in a 3-year-old girl: A case report

• Autor(a) principal: Kitakawa, Dárcio [UNESP]
• Data de Publicação: 2009
• Outros Autores: Fontes, Patrícia Campos [UNESP], Magalhães, Fernando Augusto Cintra [UNESP], Almeida, Janete Dias [UNESP], Cabral, Luiz Antonio Guimarães [UNESP]
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UNESP
• Texto Completo: http://dx.doi.org/10.1186/1752-1947-3-116; http://hdl.handle.net/11449/71316
• topic: aciclovir; anamnesis; blister; case report; child; dentistry; eye disease; female; herpes zoster; human; hypodontia; incontinentia pigmenti; mouth examination; neurologic disease; orthodontics; panoramic radiography; physical examination; preschool child; priority journal; prosthesis; skin disease; strabismus; tooth malformation

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. © 2009 Kitakawa et al; licensee BioMed Central Ltd.