title: Extranodal Rosai-Dorfman disease: involvement of eye, nose and trachea.
abstract: Rosai-Dorfman disease (RDD) is a rare non-neoplastic histiocytic proliferative disorder characterized by painless lymphadenopathy. Extranodal lesions frequently occur in the head and neck regions. We report the clinical and histological features of extranodal RDD in a 43-year-old man with a previously unreported combination of multiple gross anterior epibulbar nodules in the right eye, submucosal masses of nasal septum and trachea, and no lymphadenopathy during the 12-year follow-up. The patient underwent ophthalmological, otolaryngological and systemic evaluation; gallium 67 scintigraphy; bronchoscopy; ophthalmic ultrasound; head and neck CT scan; biopsies of epibulbar, nasal and tracheal tissues; and septoplasty. Histological specimens showed lymphocytophagocytosis and positive immunoperoxidase staining for S100 protein in foamy histiocytes; both features were typical for RDD. No response to topical or systemic steroids or to radiation therapy was recorded. Removal of nasal septum masses resolved nasal obstruction. The diagnosis of RDD requires histological and, in challenging cases, immunohistological specimens and is difficult--especially with pure extranodal localizations as in our case. RDD should be suspected in cases of subconjunctival mass and/or submucosal nasal and tracheal swellings not responding to systemic steroids.
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Padua University, Padua, Italy. gianottaviano@inwind.it
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Ottaviano
authors.forename: Daniele
authors.initials: D
authors.lastname: Doro
authors.forename: Gino
authors.initials: G
authors.lastname: Marioni
authors.forename: Pierfrancesco
authors.initials: P
authors.lastname: Mirabelli
authors.forename: Rosario
authors.initials: R
authors.lastname: Marchese-Ragona
authors.forename: Sofia
authors.initials: S
authors.lastname: Tognon
authors.forename: Filippo
authors.initials: F
authors.lastname: Marino
authors.forename: Alberto
authors.initials: A
authors.lastname: Staffieri
mesh_terms: D000328:Adult; D001706:Biopsy; D003228:Conjunctiva / Q000473:pathology / Q000601:surgery; D003229:Conjunctival Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D003952:Diagnostic Imaging; D006644:Histiocytes / Q000473:pathology; D015618:Histiocytosis, Sinus / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007124:Immunoenzyme Techniques; D008214:Lymphocytes / Q000473:pathology; D008264:Macrophages / Q000473:pathology; D008297:Male; D009300:Nasal Septum / Q000473:pathology / Q000601:surgery; D009668:Nose Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D010587:Phagocytosis / Q000502:physiology; D010950:Plasma Cells / Q000473:pathology; D009418:S100 Proteins / Q000032:analysis; D014132:Trachea / Q000473:pathology / Q000601:surgery; D014133:Tracheal Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery
chemical_list: D009418:S100 Proteins