Rosai-Dorfman Disease Revealed in the Upper Airway: a Case Report and Review of the Literature

Rosai-Dorfman disease (RDD) is a systemic disease and its etiology is not well understood. It is a very rare but established disease. We report a case of RDD diagnosed in the upper airway that became a life-threatening condition because of the stricture of the subglottic space. A 49-year-old female complained of nasal obstruction, hoarseness and lacrimation. After evaluation following by fiberscopic examination and CT scanning of the head and neck, sinusitis and laryngeal masses were detected. Endoscopic sinus surgery and endolaryngeal microsurgery were performed simultaneously. The masses in the nasal cavity and larynx showed the same histological findings. Proliferative large histiocytes with voluminous clear cytoplasm and rounded nuclei, and lymphophagocytosis known as emperipolesis are the characteristic features of RDD. In this case, extranodal manifestations in the subglottic space gradually emerged postoperatively. Tracheotomy was performed to ensure an airway and prevent suffocation. An extensive treatment is not required in most patients with RDD, however, some patients who have extranodal manifestations involving the airway may detoriorate into a life-threatening condition.