Unveiling the enigma: Navigating extranodal Rosai-Dorfman disease in the maxilla - A teen’s journey

Rosai-Dorfman disease (RDD), a rare histiocytic disorder, typically manifests as widespread lymphadenopathy. We present a unique case of extranodal RDD in a 14-year-old with a solitary maxillary lesion and mild bilateral submandibular lymphadenopathy. Clinical, radiological, and histopathological assessments confirmed RDD, highlighting the importance of a comprehensive approach. Immunohistochemistry, including CD68, CD45, CD 1a, and S100, played a crucial role in diagnosis. Differential diagnoses encompassed Langerhans cell histiocytosis, Erdheim-Chester disease, lymphomas, and histiocytic sarcoma, necessitating meticulous evaluation. Surgical excision was performed due to bone involvement, leading to successful healing in six months. Our case underscores the significance of a multidisciplinary and scientific approach for accurate RDD diagnosis and management, especially in atypical intraoral presentations.