Oral juvenile xanthogranuloma: an unusual presentation in an adult subject and a systematic analysis of published cases

Abstract Objective The purpose of this study was to describe an unusual case of intraoral juvenile xanthogranuloma (JXG) and to perform a systematic review to investigate the available literature regarding oral JXGs. Study design We present a new case of oral JXG arising in a 36-year-old Italian woman and conducted a systematic literature review in PubMed, Web of Science and Scopus, according to the PRISMA guidelines. Results Our review of the English-language literature yielded 34 cases of oral JXG, which are included in our case report. Conclusions JXG is a non-Langerhans cell histiocytosis. Oral JXG has been reported, but it is a rare manifestation. Because of the rarity of oral lesions and possible variations in the clinical and histological presentation, the correct diagnosis can be challenging, requiring a careful clinical and histopathological evaluation with adjuvant immunohistochemical studies.