Dental Management in Children with Incontinencia Pigmenti (Bloch-Sulzberger Syndrome): Case Reports and Literature Review

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.