Extranodal Rosai–Dorfman Disease With Concurrent Sinonasal and Subglottic Involvement in a 39-Year-Old Female: A Rare Presentation

Rosai–Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis that typically presents with nodal disease in young males. Extranodal involvement occurs less frequently and may involve the nasal cavity, paranasal sinuses, or orbit. Airway involvement is particularly uncommon, and simultaneous sinonasal and subglottic disease is exceptionally rare. We describe a 39-year-old female presenting with progressive nasal obstruction, throat fullness, and hoarseness. Nasal endoscopy demonstrated bilateral polypoidal tissue, while flexible laryngoscopy revealed a posterior subglottic nodule. Computed tomography showed diffuse sinonasal disease and a subglottic soft-tissue lesion without bony erosion. Histopathology confirmed RDD through characteristic emperipolesis and an S100+/CD68+/CD1a– immunophenotype. Treatment with systemic corticosteroids resulted in rapid improvement with near-complete resolution of both lesions. This case presents a highly unusual anatomical distribution of RDD with significant clinical teaching value, representing the first such report in Saudi Arabia. Awareness of this presentation may prevent misdiagnosis and unnecessary surgical intervention.