Citation: Meriem Nait Melek, Manel Chalbi, Wiem Zouaoui, Med Ali Chamli (2023). Dental Management in Children with Incontinencia Pigmenti (Bloch-Sulzberger Syndrome): Case Reports and Literature Review. Saudi J Oral Dent Res, 8(10): 313-318. 313 Saudi Journal of Oral and Dental Research Abbreviated Key Title: Saudi J Oral Dent Res ISSN 2518-1300 (Print) | ISSN 2518-1297 (Online) Scholars Middle East Publishers, Dubai, United Arab Emirates Journal homepage: https://saudijournals.com Case Report Pediatric Dentistry Dental Management in Children with Incontinencia Pigmenti (Bloch- Sulzberger Syndrome): Case Reports and Literature Review Meriem Nait Melek 1* , Manel Chalbi 2 , Wiem Zouaoui 1 , Med Ali Chamli 3 1 Resident, Pediatric Dentistry Department, Hospital La Rabta, Tunisia, Laboratory of Research ABCDF, Faculty of Dental Medicine of Monastir, Tunisia 2 Assitant Professor, Pediatric Dentistry Department, Hospital La Rabta, Tunisia, Laboratory of Research ABCDF, Faculty of Dental Medicine of Monastir, Tunisia 3 Professor, Pediatric Dentistry Department, Hospital La Rabta, Tunisia, Laboratory of Research ABCDF, Faculty of Dental Medicine of Monastir, Tunisia DOI: 10.36348/sjodr.2023.v08i10.002 | Received: 17.04.2023 | Accepted: 31.05.2023 | Published: 06.10.2023 *Corresponding author: Meriem Nait Melek Resident, Pediatric Dentistry Department, Hospital La Rabta, Tunisia, Laboratory of Research ABCDF, Faculty of Dental Medicine of Monastir, Tunisia Abstract Introduction: Incontinentia Pigmenti (IP) is a multisystem genodermatosis characterized by cutaneous, neurologic, ophthalmologic, and dental abnormalities. Skin alterations are the main characterestic of IP. They can change and even disappear over time, in contrast, dental ones are permanent and considered as the most frequent extra cutaneous signs. The aim of this work is to present two rare clinical reports of Incontinentia Pigmenti and to adress its frequent orofacial manifestations in the dental litterature. Case Reports: In this paper, we report two clinical cases of incontinencia pigmenti in two female patients, five and nine years old, who were first examined by a dermatologist due to skin changes and then referred to our pediatric dentistry department in la Rabta hospital for oral examination. We managed these cases in collaboration with the pediatric department. We also conducted a research in the following electronic databases: PubMed and google scholar using the following keywords: ("Incontinentia Pigmenti" AND "Child" AND "Tooth Abnormalities”), from 2011 to 2021, to compare dental findings in our patients with literature. Discussion: The most representative dental features of IP are: delayed tooth eruption, agenesis, high arched palate, peg like and malformed teeth, affecting both primary and permanent teeth. Therapeutic management of our patients consisted in the replacement of absent teeth with an aesthetic and functional space maintainer. The assessment of orofacial manifestations in Children with Bloch Sulzberger syndrome indicates that it appears almost exclusively in females and is usually lethal in males. The clinical expression of this disease found in our patients reflects the Data reported in the literature. The latter showed that the presence of symptoms other than skin changes is important if dermatological signs are subtle, which is in accordance with both our patients’ clinical expression. The most relevant finding of this review is the observation that most IP patients with odontological findings had the congenital absence of six or more teeth opposed to our second patient who had only one absent tooth. Conclusions: Incontinencia pigmenti is of a good prognosis but it may affect patients' quality of life hence the importance of knowing this syndrom and its oro-facial characteristics in order to obtain a correct diagnosis and apply an appropriate approach treatment. Keywords: Bloch-sulzberger, rare syndrom, dermatologic, oral manifestations, dental, management. Copyright © 2023 The Author(s): This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC BY-NC 4.0) which permits unrestricted use, distribution, and reproduction in any medium for non-commercial use provided the original author and source are credited. INTRODUCTION Incontinentia Pigmenti (IP), also known as « Bloch-sulzberger » disease, is an X-linked dominant syndrom predominantly affecting females and lethal in males because of x-chromosome dizygosity and negative selection of cells carrying the mutant x chromosome. It’s a rare genodermatosis with an incidence of 0.7 per 100.000 births [1, 2]. This multisystem disease is characterized by cutaneous, neurologic, ophthalmologic and dental abnormalities. Skin alterations are the main characterestic of IP. These manifestations can change