Dental and oral anomalies in incontinentia pigmenti: a systematic review

  title={Dental and oral anomalies in incontinentia pigmenti: a systematic review},
  author={Sne{\vz}ana Mini{\'c} and Du{\vs}an Trpinac and Heinz Gabriel and Martin Gen{\vc}{\'i}k and Miljana Obradovi{\'c}},
  journal={Clinical Oral Investigations},
ObjectivesIncontinentia 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 methodsWe analyzed the literature data from 1,286 IP cases from the period 1993–2010.ResultsDental and/or oral anomalies were diagnosed for 54.38% of the investigated… 
A Report of Incontinentia Pigmenti in an 11-year-old Girl
A removable space maintainer was constructed for a female patient, aged 11 years, presenting with dental manifestations such as hypodontia, conical teeth, delayed tooth eruption, narrow and atrophic dental ridge, and also some non-dental findings of IP.
Orodental manifestations in ectodermal dysplasia—A review
  • B. Bergendal
  • Medicine
    American journal of medical genetics. Part A
  • 2014
There is a clinical overlap in oral signs and symptoms between isolated oligodontia and the most common EDs, and studies with genetically confirmed diagnoses and larger cohorts would create a basis for refined diagnostics, where oral examinations should be an integrated part of clinical assessment.
Intrafamilial clinical variability in four families with incontinentia pigmenti
This study was the first to address the variability of dental defects within IP families and contributes to the understanding of the genetic background involved in IP expressivity.
Dental anomalies in 14 patients with IP: clinical and radiological analysis and review
This study shows that patients with IP experience significant loss of teeth, especially in permanent dentition, and have an increased risk of high-arched palate compared to the general population.
Systematic review of central nervous system anomalies in incontinentia pigmenti
The frequency of CNS anomalies, similar to the frequency of retinal anomalies in IP patients, concurrent with their severity, supports their recognition in the list of IP minor criteria.
Incontinentia Pigmenti: A Comprehensive Review and Update.
An updated review of the literature, highlighting diagnosis, epidemiology, pathophysiology, clinical features, and management ofcontinentia pigmenti, a rare syndrome with skin lesions, ocular abnormalities in the retina and elsewhere, central nervous system abnormalities, and teeth defects.
Dental treatment considerations for a pediatric patient with incontinentia pigmenti (Bloch-Sulzberger syndrome)
Early and longitudinal involvement of pediatric dentist to deal with the dental complications of IP can not only solve the esthetic problem and oral function but also maintain the oral health of children with IP to adulthood.
Incontinentia pigmenti diagnostic criteria update
In the diagnosis of IP, the presence of IKBKG mutation typical for IP, and existence of family relatives with diagnosed IP are taken into account and proposed as major criteria one of the stages of IP skin lesions.
First IKBKG gene mutation study in Serbian incontinentia pigmenti patients.
It is demonstrated that molecular genetic testing which specifically detects the common IKBKG mutations, the only known cause of IP, is useful in diagnosing IP especially in mild or atypical cases.


Clinical features of incontinentia pigmenti with emphasis on oral and dental abnormalities
This is the first time that the presence of gothic palate in patients with IP has been documented and, in two female subjects and one male subject, in which nonrandomed X inactivation did not occur, gothsic palate could be supposed as characteristic of IP.
Ocular anomalies in incontinentia pigmenti: literature review and meta-analysis.
This study suggests that IP is far more frequent than anyone could estimate and early detection and treatment of ophthalmological, neurological etc. findings may prevent severe consequences that IP may cause.
Dental defects in incontinentia pigmenti: case report.
A 12-year-old female with incontinentia pigmenti associated with enamel hypocalcification and hypoplasia of the permanent teeth is reported, one of the lesser known ectodermal dysplasias with fewer than 800 cases reported in the world literature.
Clinical study of 40 cases of incontinentia pigmenti.
The distribution of manifestations in a pediatric cohort and guidelines for follow-up of incontinentia pigmenti are analyzed to better understand the pathological mechanisms of IP and develop new therapies.
Incontinentia pigmenti: a case report.
The oral and dental manifestations of an IP case in a Venezuelan pediatric patient showed macular pigmented lesions in her face, trunk, back, legs, and fingers as well as abnormal hair distribution, alopecia on the vertex, and hypoplasia of eyebrows.
Incontinentia pigmenti in a surviving male is accompanied by hypohidrotic ectodermal dysplasia and recurrent infection.
A NEMO stop codon mutation has been identified that has arisen de novo in his affected mother and may explain why this child survived for two years and 7 months with familial Incontinentia pigmenti.
Incontinentia pigmenti: seven cases with dental manifestations.
A large case series of seven patients, all female, who presented to the Department of Paediatric Dentistry at the Eastman Dental Hospital over the last 16 years is reported and the dental features of these cases were typical and included missing teeth, microdontia and delayed eruption.
Incontinentia pigmenti case series: clinical spectrum of incontinentia pigmenti in 53 female patients and their relatives
It is found that, while the frequency of the first three cutaneous stages of IP was comparable with previous studies, none of the secondary cases manifested any serious neurological complications but all displayed stage IV pale anhidrotic reticulate lines on their posterior calves.
Incontinentia pigmenti: a review and update on the molecular basis of pathophysiology.
The mechanism by which NEMO/IKKgamma deficiency causes, via the NF-kappaB pathway, the phenotypical expression of the disease has recently been elucidated, and the newest research findings on eosinophil recruitment through eotaxin release by activated keratinocytes are described in the review.