Dental and oral anomalies in incontinentia pigmenti: a systematic review
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- Minić, S., Trpinac, D., Gabriel, H. et al. Clin Oral Invest (2013) 17: 1. doi:10.1007/s00784-012-0721-5
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Incontinentia pigmenti (IP) is an X-linked genodermatosis caused by a mutation of the IKBKG gene. The objective of this study was to present a systematic review of the dental and oral types of anomalies, to determine the total number and sex distribution of the anomalies, and to analyze possible therapies.
Materials and methods
We analyzed the literature data from 1,286 IP cases from the period 1993–2010.
Dental and/or oral anomalies were diagnosed for 54.38% of the investigated IP patients. Most of the anomaly types were dental, and the most frequent of these were dental shape anomalies, hypodontia, and delayed dentition. The most frequent oral anomaly types were cleft palate and high arched palate. IKBKG exon 4–10 deletion was present in 86.36% of genetically confirmed IP patients.
According to the frequency, dental and/or oral anomalies represent the most frequent and important IP minor criteria. The most frequent mutation was IKBKG exon 4–10 deletion. The majority of dental anomalies and some of the oral anomalies could be corrected.
Because of the presence of cleft palate and high arched palate in IP patients, these two anomalies may be considered as diagnostic IP minor criteria as well.
KeywordsIncontinentia pigmentiIKBKG geneDental anomaliesOral anomaliesPalate anomaliesSystematic review
Inhibitor of kappa B kinase gamma
NF-κB essential modulator
Nuclear factor-kappa B