Abstract

Rosai-Dorfman disease is a rare cause of lymphadenopathy in adults. Extranodal presentation of the disease is possible mainly in the head and the neck region.The diagnosis is based on histological examination with the presence of histiocytes, which are S-100 positive, CD68 positive, and CD1a negative immunohistochemistry.The outcome is usually good in asymptomatic forms of the disease with no critical organ involved. The surgical resection is appropriate to the localised symptomatic form of the disease while corticosteroids are indicated in disseminated RDD as a first-line therapy.Inspired by our case, rare localisation of Rosai-Dorfman disease (RDD), led to clinical and therapeutic issues. That is why a review of the literature must be undertaken, to share experiences.

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