Rosai-Dorfman disease or sinus histiocytosis with massive lymphade nopathy (SHML)is a rare benign disorder of uncertain aetiology. The condition was first described in 1969 (Rosai and Dorfman, 1969) and a further account in 1972 (Rosai and Dorfman, 1972) described the clinical and pathological features. The disease typically presents in young people with massive, painless cervical lymphadenopathy and may be accompanied by malaise, fever and weight loss. Extranodal disease occurs in 43 percent of patients and most commonly involves the skin, nasal cavity and paranasal sinuses. The condition may present to the ear, nose and throạt (ENT) department in a number of ways but isolated salivary gland disease is relatively unusual since patients presenting with salivary gland involvement possess additional extranodal disease in the majority of cases.
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