title: Dental and oral anomalies in incontinentia pigmenti: a systematic review.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  We analyzed the literature data from 1,286 IP cases from the period 1993-2010.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: School of Medicine, University of Belgrade, Deligradska 34, 11000, Belgrade, Serbia. dtrpinac@eunet.rs
authors.forename: Snežana
authors.initials: S
authors.lastname: Minić
authors.forename: Dušan
authors.initials: D
authors.lastname: Trpinac
authors.forename: Heinz
authors.initials: H
authors.lastname: Gabriel
authors.forename: Martin
authors.initials: M
authors.lastname: Gencik
authors.forename: Miljana
authors.initials: M
authors.lastname: Obradović
mesh_terms: D000848:Anodontia / Q000209:etiology; D002972:Cleft Palate / Q000209:etiology; D005091:Exons / Q000235:genetics; D017353:Gene Deletion; D006801:Humans; D051550:I-kappa B Kinase / Q000235:genetics; D007184:Incontinentia Pigmenti / Q000150:complications*; D009056:Mouth Abnormalities / Q000209:etiology*; D010159:Palate / Q000002:abnormalities; D014071:Tooth Abnormalities / Q000209:etiology*; D014078:Tooth Eruption / Q000502:physiology
chemical_list: C491731:IKBKG protein, human; D051550:I-kappa B Kinase