Abstract
Introduction: Rosai Dorfman disease is a benign non-Langerhansian histiocytosis. Its etiology remains unknown. It consists of the presence of histiocytes within lymph nodes, but other extra-nodal localizations such as nasosinusal or orbit may be noted. It frequently affects young adults and adolescents. Diagnosis is based on anatomopathological evidence. The aim of this study was to specify the clinical and therapeutic features of this disease.
Methods: We conducted a retrospective descriptive study of 06 patients diagnosed with Rosai Dorfman disease in the ear, nose and throat (ENT) department of Habib Thameur Hospital Tunisia over a 07-years period from 2017-2023. Results: Six patients were diagnosed with Rosai Dorfman disease in our department. The mean age was 18.7, with extremes ranging from 9 months to 66 years and a sex ratio (M/F) of 1. The mean time from symptom onset to diagnosis varies from 2 months to 12 months. Two patients had single lymph node involvement, 2 had isolated nasosinusal involvement, one had nasosinusal involvement associated with pulmonary involvement, and one had lymph node involvement associated with laryngeal, parotid, cutaneous and mammary involvement. The diagnosis of certainty was made on definitive histopathological examination with immunohistochemical study. Two patients required corticosteroid therapy at a dose of 1mg/kg/d combined with an immunosuppressive drug such as methotrexate (15mg/week).
Conclusion: The diagnosis of Rosai Dorfman disease relies on histopathological examination Although rare, it is an entity that should not be ignored to ensure better therapeutic management.
Key words: Rosai-Dorfman disease, Histiocytosis, Lymphadenopathy nodal, extranodal.
