Unisystem Langerhans cell histiocytosis in maxillofacial region in pediatrics: comprehensive and systematic review

Abstract

Background: The study aimed to identify, enlist, and analyze cases of unisystem LCH in the maxillofacial pediatric population to understand the clinical presentation and encourage the consideration of this rare disease in the differential diagnosis. Langerhans cell histiocytosis (LCH) is an aggressive benign condition affecting mainly the pediatric population. It can be easily masked as periodontal disease in the maxillofacial region. Early diagnosis and a systemic evaluation are of utmost importance.

Methodology: We are presenting a complete review of literature in the pediatric population according to PRISMA guidelines for clinicopathologic, histopathological, immunohistochemistry, and treatment for unisystem LCH. The risk of bias assessment across studies was done using a Case series appraisal checklist by Guo et al. 53 RESULTS: Forty-nine articles (152 cases) were selected which met our inclusion and exclusion criteria to be included in our review. Most of the patients fall in 6-12 years of age with the involvement of the mandibular body region in 40.79% cases. This disease mainly presents as erythematous gingiva, pain, swelling, and mobile teeth. Management can range from minimal intervention to chemotherapy and surgery.

Conclusion and practical implications: Although this is a rare condition, it should be considered especially in the pediatric population with periodontitis type lesions and floating teeth and comprehensive management should be followed. Early diagnosis of the disease is very important.

Keywords: Eosinophilic granuloma; Jaws; Langerhans cell histiocytosis; Mandible; Maxilla; Pediatrics; maxillofacial.