Rosai–Dorfman Disease in the Head and Neck Region: A Case Report

Background: Rosai–Dorfman disease (RDD), also referred to as sinus histiocytosis with massive lymphadenopathy, is a rare, benign, non-Langerhans cell histiocytic proliferative disorder of uncertain etiology. Although it primarily involves lymph nodes, particularly the cervical group, extranodal manifestations occur in approximately 40% of cases, most frequently affecting the head and neck region. Case Presentation: We report a case of a 12-year-old female presenting with multiple neck swelling for 6 months with history of pulmonary tb in parent (now post ATT). Clinical history examination and radiologic findings raised suspicion of lymphoma or chronic granulomatous disease (e.g. tuberculosis). Histopathological examination revealed accumulation of histiocytes with enlarged, round to oval hypochromatic nuclei and abundant eosinophilic cytoplasm, often containing engulfed intact inflammatory cells known as emperipolesis consistent with Rosai–Dorfman disease. The patient was managed with corticosteroids with favorable outcome on follow-up. Conclusion: RDD in the ENT region is a diagnostic challenge due to its variable presentation and rarity. Histopathology remains the gold standard for diagnosis. Awareness of this condition is essential to avoid unnecessary aggressive management.