Rosai-Dorfman disease is a rare histiocytic disorder characterized by accumulation of histiocytes in the affected tissue. Herein we report on a case of Rosai-Dorfman disease with multiple lymphadenopathy and multiple extranodal lesions. A 22-year-old woman was referred to our hospital with bilateral submandibular swelling and a localized swelling in the root of the nose. Endoscopic examination revealed mass lesions in both nasal cavities and the subglottic area. A full-body CT revealed multiple nasal masses, cervical lymphadenopathy, and an intrathymic mass. We diagnosed her as having Rosai-Dorfman disease based on the histopathological findings of accumulation of histiocytes and emperipolesis in the nasal lesion and cervical lymph node specimens. Histopathological examination is required for the diagnosis of Rosai-Dorfman disease. After steroid treatment for 3 months, reduction in the size of the lymph nodes in the neck, nasal cavity lesions, and intrathymic mass was noted. However, the size of the subglottic mass remained almost unchanged. In this case, steroid treatment was mostly effective on the lesions. In case of airway obstruction due to increase in the size of the subglottic mass, surgical resection should be indicated.
