Abstract
Rosai-Dorfman disease is a rare histiocytic disorder of unknown etiology. This study aims to report an uncommon case of the disease in the submandibular region. A 7-year-old female patient presented with submandibular swelling of 3 months' duration. No intraoral manifestations were observed. Computed tomography scan demonstrated reabsorption of the mandibular cortical bone. An incisional biopsy was performed, and microscopic examination showed a diffuse mononuclear cell infiltration, with xanthomatous cells and emperipolesis of the lymphocytes. Immunohistochemistry was positive for LCA, CD3, CD20, VS38C, CD68, and S100 and the Ki-67 index was high, leading to the diagnosis of Rosai-Dorfman disease. Blood count evidenced anemia. Other affected sites were discarded by bone scintigraphy. Corticoid therapy with prednisone 40 mg for 6 months was prescribed. The patient was lost to follow-up. Rosai-Dorfman disease involving gnathic bones is rare and requires early diagnosis due to the possibility of spontaneous remission and conservative treatment.
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