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Published in English in Tropical Dental Journal Volume 45 - December 2022 pages 5-9
Authors : S. Dhaoui, A. Sari, J. Zaroui, M. Chalbi, I. Jazi, M. Chemli - Tunisia
Le Syndrome de Letterer-Siwe (SLD) est une maladie rare qui touche les jeunes enfants de moins de 3 ans, sa prévalence est estimée à 1/50.0000.
Il est caractérisé par une migration et une prolifération monoclonale de cellules dendritiques spécifiques. Elle est considérée comme la forme multifocale agressive et multi-systémique de l’Histiocytose à Cellules de Langerhans (HCL). Elle affecte principalement les os et la peau ; les lésions osseuses se produisent surtout dans le crâne, les os longs et la mandibule, mais peuvent toucher d’autres organes. Le pronostic n’est pas favorable dans la plupart des cas de la maladie de Letterer-Siwe, le décès pouvant survenir pendant la vie intra-utérine ou quelques semaines après la naissance.
En connaissant les symptômes bucco-dentaires courants de la maladie, les dentistes pédiatriques peuvent jouer un rôle essentiel dans le diagnostic du syndrome de Letterer-Siwe à un stade précoce et prévenir l’extension de la maladie en adressant le jeune patient à un spécialiste.
Le but de cet article était de décrire les manifestations orales de la maladie de Letterer-Siwe à travers deux cas référés au département de dentisterie pédiatrique de l’hôpital La Rabta, Tunisie.
Letterer-Siwe Syndrome (LSD) is a rare disease that affects young children under the age of 3 years old, its prevalence is estimated at 1/50.0000.
It is characterized by monoclonal migration and proliferation of specific dendritic cells. It is considered to be the aggressive multifocal multisystem form of the Langerhans‘ Cell Histiocytosis (LCH). It affects primarily the bones and skin; Bone lesions occur mainly in the skull, long bones and mandible but might involve other organs. The prognosis is not favorable in most cases of the Letterer-Siwe disease, death might occur during intrauterine life or within a few weeks of birth.
By knowing the common oral symptoms of the disease, pediatric dentists can play a vital role in the diagnosis of Letterer-Siwe Syndrome at an early stage preventing the extension of the disease by addressing the young patient to a specialist.
The aim of this paper was to describe the oral manifestations of Letterer-Siwe disease through two cases referred to the department of pediatric dentistry in La Rabta hospital, Tunisia.
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