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Dental anomalies in 14 patients with IP: clinical and radiological analysis and review

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Abstract

Objectives

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.

Clinical significance

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.

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Abbreviations

IP:

Incontinentia pigmenti

IKBKG :

Inhibitor of kappa B kinase gamma

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Acknowledgments

We would like to thank the CAPES Foundation and the Postgraduate Program of the Federal University of Health Sciences of Porto Alegre (UFCSPA). We also thank Professor of Statistics, Ceres Andrade, for helping in the development of this work.

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Authors and Affiliations

  1. Postgraduate Program in Pathology, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil

    Fernanda D. Santa-Maria & Cláudia S. Poziomczyk

  2. Postgraduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Luiza Monteavaro Mariath

  3. Departamento de Fonoaudiologia, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil

    Marcia A. P. Maahs

  4. Santa Casa de Misericórdia de Porto Alegre Hospital, Porto Alegre, RS, Brazil

    Marcia A. P. Maahs, Rafael F. M. Rosa, Paulo R. G. Zen & Ana Elisa Kiszewski

  5. Serviço de Genética Médica, Departamento de Clínica Médica, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil

    Rafael F. M. Rosa & Paulo R. G. Zen

  6. Departamento de Genética, Instituto de Biociências, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil

    Lavínia Schüller-Faccini

  7. Serviço de Dermatologia, Departamento de Clínica Médica, Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil

    Ana Elisa Kiszewski

Authors
  1. Fernanda D. Santa-Maria
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  2. Luiza Monteavaro Mariath
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  3. Cláudia S. Poziomczyk
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  4. Marcia A. P. Maahs
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  7. Lavínia Schüller-Faccini
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  8. Ana Elisa Kiszewski
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Corresponding author

Correspondence to Ana Elisa Kiszewski.

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Conflict of interest

The authors declare that they have no conflict of interest.

Funding

Fernanda Santa Maria received CAPES scholarship to conduct this study. No additional grants or fundings were received.

Ethical approval

All procedures in this study were in accordance with the ethical standards of the institution (CEP-UFCSPA) and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. All were briefed on the technical and scientific background relevant to the research project. Informed consent was obtained from all individual participants included in the study.

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Santa-Maria, F.D., Mariath, L.M., Poziomczyk, C.S. et al. Dental anomalies in 14 patients with IP: clinical and radiological analysis and review. Clin Oral Invest 21, 1845–1852 (2017). https://doi.org/10.1007/s00784-016-1977-y

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