AI Article Synopsis
- Incontinentia pigmenti, or Bloch-Sulzberger syndrome, is a rare X-linked dominant disorder predominantly affecting women, with symptoms impacting the skin, eyes, hair, central nervous system, and teeth.
- A case of a 3-year-old girl illustrates how her skin and eye issues were less severe compared to the significant dental problems, particularly hypodontia (missing teeth).
- The findings highlight the necessity for a collaborative approach in dental treatment, integrating pediatric dentistry, orthodontics, and prosthetics, as dental anomalies are key for diagnosis and management of the condition.
Article Abstract
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.
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|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2783057 | PMC |
| http://dx.doi.org/10.1186/1752-1947-3-116 | DOI Listing |
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