Preview

Pediatric dentistry and dental prophylaxis

Advanced search

Oral health status of a patient with hypomelanosis of Ito: a case report

https://doi.org/10.33925/1683-3031-2025-865

Abstract

Relevance. The etiology of hypomelanosis of Ito is associated with dysfunction of neural crest cells. Therefore, this genetic disorder may be directly associated with craniofacial skeletal and dental anomalies. Early diagnosis and timely initiation of treatment are crucial for effective management of the condition.

Clinical case description. This article presents a clinical case describing the oral manifestations of hypomelanosis of Ito in an 8.5-year-old child. The patient was referred to the clinic of Kuban State Medical University, Ministry of Health of the Russian Federation, with complaints of extensive crown defects in both primary and permanent molars, abnormal morphology of anterior teeth, and congenital absence of several teeth. The diagnosis of hypomelanosis of Ito was established by medical geneticists based on a constellation of clinical features. The patient had previously received dental care. A comprehensive treatment plan was developed, including oral rehabilitation and orthodontic management.

Conclusion. Owing to the rarity of this genetic neurocutaneous syndrome, only a limited number of cases have been documented in the literature, with oral manifestations reported in isolated instances. The diagnostic complexity associated with such conditions highlights the importance of a comprehensive evaluation of craniofacial and dental features as part of the clinical assessment.

About the Authors

O. A. Pavlovskaya
Kuban State Medical University
Russian Federation

Olga A. Pavlovskaya, DMD, PhD, Associate Professor, Department of Pediatric Dentistry, Orthodontics and Maxillofacial Surgery

Krasnodar



M. N. Mitropanova
Kuban State Medical University
Russian Federation

Marina N. Mitropanova, DMD, PhD, DSc, Head of the Department of Pediatric Dentistry, Orthodontics and Maxillofacial Surgery

Krasnodar



V. V. Volobuev
Kuban State Medical University
Russian Federation

Vladimir V. Volobuev, DMD, PhD, Associate Professor, Department of Pediatric Dentistry, Orthodontics and Maxillofacial Surgery

4 Mitrofan Sedin Str., Krasnodar, 350053



L. F. Tereschenko
Kuban State Medical University
Russian Federation

Lyudmila F. Tereshenko, DMD, Assistant Professor, Department of the General Dentistry

Krasnodar



A. V. Burdina
Kuban State Medical University
Russian Federation

Alisa V. Burdina, 5th year student, Dental School

Krasnodar



A. R. Gazarova
Kuban State Medical University
Russian Federation

Aleksandra R. Gazarova, DMD, Resident, Department of the General Dentistry

Krasnodar



N. A. Yevdokimova
Kuban State Medical University
Russian Federation

Nina A. Yevdokimova, DMD, Assistant Professor, Department of the General Dentistry

Krasnodar



K. K. Gasparyan
Kuban State Medical University
Russian Federation

Karen K. Gasparyan, director, dentist, “Аuthor's dentistry Dr. Gasparyan K”

Krasnodar



References

1. Yusupova LA, Yunusova EI, Garaeva ZS, Mavlyutova GI. Phacomatoses: diagnosis, clinic and features of the course of various forms of the disease. Lechaschi Vrach. 2018;(5):35-40 (In Russ.). Available from: https://elibrary.ru/item.asp?id=35112481

2. Gorken F, Goksen I, Erdem A, Kuru S, Sepet E. Multiple talon cusps, dens in dente and concrescence in a child with hypomelanosis of Ito. Open Journal of Stomatology. 2012;(2):206-209. doi: 10.4236/ojst.2012.23037

3. Kosheleva ML, Efet EA, Laptiev SA. Hypomelanosis of Ito: description of the clinical case. The Scientific Notes of the Pavlov University. 2023;30(2):61-68. (In Russ.) doi:10.24884/1607-4181-2023-30-2-61-68

4. Davydova MA, Santalova GV, Gasilina ES, Stadler ER, Shorokhov SE. Clinical case of hypomelanosis Ito diagnostics. Izvestia of Samara Scientific Center of the Russian Academy of Sciences. 2015;17(5–2):351–354. (In Russ.). Available from: https://elibrary.ru/item.asp?id=26092386

5. Olisova OY, Grabovskaya OV, Teplyuk NP, Belousova TA, Varshavsky VA, Arsentyev NS, et al. Unilateral dermatoses: Relationship with Blashko's lines and neurological changes. Russian Journal of Skin and Venereal Diseases. 2014;17(5):22–29 (In Russ.). doi: 10.17816/dv36898

6. Demartini Z Jr, Teixeira BCA, Koppe GL. Carotid Artery Aneurysm and Hypomelanosis of Ito. Pediatr Neurosurg. 2023;58(2):114-116. doi: 10.1159/000530354

7. Kotov, A.S. Genetically determined and other specific forms of epilepsy occurring in childhood: An educational and methodological guide / A.S. Kotov, K.V. Firsov // – Moscow: State Medical University of the Moscow State Medical University named after M.F. Vladimirsky (In Russ.). eLIBRARY ID: 50514173.

8. Mironov MB, Borovikova NYu, Borovikov KS, Mukhin KYu. Hypomelanosis of Ito with epilepsy: two cases in clinical practice. Epilepsy and Paroxysmal Conditions. 2012;4(1):8–12 (In Russ.). Available from: https://elibrary.ru/item.asp?id=17898750

9. Hasegawa T, Akazawa Y, Kitamura T, Sugimoto A, Ueda K, Iwamoto T. Dental findings and management in a child with hypomelanosis of Ito. Pediatric Dental Journal. 2014;24 (3):173–177. doi: 10.1016/j.pdj.2014.08.002

10. Gupta M, Gupta P, Sharma R, Gupta A. Hypomelanosis of Ito with gynaecomastia and dental anomaly. BMJ Case Rep. 2018;2018:bcr2018225055. doi: 10.1136/bcr-2018-225055

11. Bodemer C. Incontinentia pigmenti and hypomelanosis of Ito. Handb Clin Neurol. 2013;111:341-347. doi: 10.1016/B978-0-444-52891-9.00040-3

12. Kentab AY, Hassan HH, Hamad MH, Alhumidi A. The neurologic aspects of hypomelanosis of Ito: Case report and review of the literature. Sudan J Paediatr. 2014;14(2):61-70. Available from: https://pubmed.ncbi.nlm.nih.gov/27493406/

13. Nistal M, Paniagua R, González-Peramato P, Reyes-Múgica M. Perspectives in Pediatric Pathology, Chapter 11. Testicular Pathology of Hamartomatous Origin. Pediatr Dev Pathol. 2016;19(1):1-11 doi: 10.2350/14-04-1472-PB.1

14. Lone P, Kour I, Gandral A. Intra Oral Approach for Complex & Compound Odontomas (Large or Small). Modern Plastic Surgery. 2014;(4):35–45. doi: 10.4236/mps.2014.43008


Supplementary files

Review

For citations:


Pavlovskaya O.A., Mitropanova M.N., Volobuev V.V., Tereschenko L.F., Burdina A.V., Gazarova A.R., Yevdokimova N.A., Gasparyan K.K. Oral health status of a patient with hypomelanosis of Ito: a case report. Pediatric dentistry and dental prophylaxis. 2025;25(1):102-108. (In Russ.) https://doi.org/10.33925/1683-3031-2025-865

Views: 154


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 1683-3031 (Print)
ISSN 1726-7218 (Online)