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Clinical Problem Solving: Radiology
March 2009

Radiology Quiz Case 2: Diagnosis

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2009;135(3):316-319. doi:10.1001/archoto.2008.552-b

Rosai-Dorfman disease, a generally benign entity characterized by sinus histiocytosis with massive lymphadenopathy, was first described by Rosai and Dorfman1in 1969. The disease, which occurs primarily in children and young adults, mostly presents with massive cervical lymphadenopathy (90%), fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia.2When it affects nonlymphoid organs, it is classified as extranodal Rosai-Dorfman disease. Extranodal disease occurs in 43% of patients, and 75% of the extranodal lesions appear in the head and neck region.2Orbital involvement, which is characterized by exophthalmos, ptosis, conjunctival injection, diplopia, and dry eyes,3has been reported in 11% of the extranodal cases. Concomitant involvement of the orbit and nasal cavity has been documented in very few reports.4,5

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