Clinical Oral Investigations

, Volume 17, Issue 1, pp 1-8

First online:

Dental and oral anomalies in incontinentia pigmenti: a systematic review

  • Snežana MinićAffiliated withSchool of Medicine, University of BelgradeInstitute of Dermatology, Clinical Center of Serbia Email author 
  • , Dušan TrpinacAffiliated withInstitute of Histology and Embryology, School of Medicine, University of Belgrade
  • , Heinz GabrielAffiliated withCenter for Medical Genetics, Diagenos
  • , Martin GencikAffiliated withCenter for Medical Genetics, Diagenos
  • , Miljana ObradovićAffiliated withInstitute of Histology and Embryology, School of Medicine, University of Belgrade

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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.

Clinical relevance

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.


Incontinentia pigmenti IKBKG gene Dental anomalies Oral anomalies Palate anomalies Systematic review