title: Dental anomalies in 14 patients with IP: clinical and radiological analysis and review.
abstract: OBJECTIVE
  Current knowledge on dental anomalies in patients with incontinentia pigmenti (IP) has been obtained by examining case reports; however, an overall characterization of such alterations remains lacking. The objective of this study was to determine the frequency, type and location of dental alterations in IP using a case series.
  
  
  METHODS
  Fourteen patients (9 children and 5 adults) with a clinical diagnosis of IP who presented dental anomalies were included in this study. All patients were administered a clinical questionnaire, dental examination and radiological investigation.
  
  
  RESULTS
  In the present case series, agenesis of primary dentition was present in 60 % of patients and agenesis of permanent tooth was present in 92.8 % of patients. Most cases were missing at least 6 teeth. Second molar agenesis was present in 13 patients (92.8 %). Anomalies in dental crowns occurred in 71.4 % of cases, and the central incisor was most frequently affected. Two adult patients still had primary teeth. Malocclusion was found in 10 patients (71.4 %). High-arched palate was observed in 7 (50 %) patients.
  
  
  CONCLUSIONS
  Patients with IP present alterations in both primary and permanent dentition. Because the agenesis of permanent teeth is more common, primary teeth are not always replaced. In addition, the durability of primary dentition appears to be greater in IP.
  
  
  CONCLUSIONS
  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. Prophylactic care of primary teeth in IP is relevant for improving functional and aesthetic outcomes until dental prostheses are implanted.
authors.affiliation: Postgraduate Program in Pathology, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
authors.forename: Fernanda D
authors.initials: FD
authors.lastname: Santa-Maria
authors.affiliation: Postgraduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
authors.forename: Luiza Monteavaro
authors.initials: LM
authors.lastname: Mariath
authors.affiliation: Postgraduate Program in Pathology, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
authors.forename: Cláudia S
authors.initials: CS
authors.lastname: Poziomczyk
authors.affiliation: Departamento de Fonoaudiologia, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.|Santa Casa de Misericórdia de Porto Alegre Hospital, Porto Alegre, RS, Brazil.
authors.forename: Marcia A P
authors.initials: MAP
authors.lastname: Maahs
authors.affiliation: Santa Casa de Misericórdia de Porto Alegre Hospital, Porto Alegre, RS, Brazil.|Serviço de Genética Médica, Departamento de Clínica Médica, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
authors.forename: Rafael F M
authors.initials: RFM
authors.lastname: Rosa
authors.affiliation: Santa Casa de Misericórdia de Porto Alegre Hospital, Porto Alegre, RS, Brazil.|Serviço de Genética Médica, Departamento de Clínica Médica, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
authors.forename: Paulo R G
authors.initials: PRG
authors.lastname: Zen
authors.affiliation: Departamento de Genética, Instituto de Biociências, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
authors.forename: Lavínia
authors.initials: L
authors.lastname: Schüller-Faccini
authors.affiliation: Santa Casa de Misericórdia de Porto Alegre Hospital, Porto Alegre, RS, Brazil. kiszewski@gmail.com.|Serviço de Dermatologia, Departamento de Clínica Médica, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil. kiszewski@gmail.com.
authors.forename: Ana Elisa
authors.initials: AE
authors.lastname: Kiszewski
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications* / Q000000981:diagnostic imaging; D008297:Male; D011862:Radiography, Panoramic; D011795:Surveys and Questionnaires; D014071:Tooth Abnormalities / Q000150:complications* / Q000000981:diagnostic imaging
keywords: Dental anomalies; Ectoderm anomalies; Genodermatosis; Hypodontia; Incontinentia pigmenti; Missing teeth