ORAL SIGNS IN A PATIENT WITH UNDIAGNOSED ROSAI-DORFMAN DISEASE: A CASE REPORT

Rosai-Dorfman disease (RDD) is a rare, nonneoplastic, idiopathic histiocytic proliferation disorder. Massive lymphadenopathy is one of the main clinical findings, although it can occur without nodal involvement, mimicking other conditions and making diagnosis difficult. Oral manifestations are extremely rare. A 65-year-old female patient was attended for evaluation in a stomatology service at a cancer hospital. Physical examination revealed a reddish macule with irregular contour and telangiectasia and a 4-cm, nodular, sessile, well-delimited lesion with normal coloration that softened to palpation. Both lesions were asymptomatic and localized in the hard palate. Furthermore, multiple papular lesions with bluish coloration in the face were observed. An incisional biopsy of both intraoral lesions was performed, and after the histopathologic and immunohistochemical analysis (S100), the diagnosis of RDD was obtained. Therapy was initially instituted with oral prednisone 60 mg/d resulting in significant regression of clinical signs. Currently, the patient is under monthly follow-up in stomatology and hematology services.