Rosai-Dorfman disease

by Sampan Singh Bist, MS, Manisha Bisht, MBBS, Saurabh Varshney, MS, and Sanjeev Kishore, MD | Tuesday, January 01, 2008

The term sinus histiocytosis with massive lymphadenopathy (SHML) was introduced by doctors Rosai and Dorfman in 1969 as a well-defined clinicopathologic entity that is now widely known as Rosai-Dorfman disease. SHML is a rare self-limited pseudolymphomatous disorder of unknown etiology usually presenting with cervical lymphadenopathy, fever, elevated erythrocyte sedimentation rate, and hematologic abnormalities. It shows a worldwide distribution with childhood predilection and has a 2:1 male-to-female ratio.Although cervical lymph node involvement is most common, axillary, inguinal, and mediastinal regions can also be affected. Extranodal manifestation of the disease is observed in 28 to 43% of cases, with the head and neck region being the most common site affected. The most frequent otorhinolaryngologic manifestations are found in the nasal cavity, followed by the pharynx, paranasal sinuses, amygdala, and trachea.The diagnosis of Rosai-Dorfman disease is confirmed by histopathologic .../continued/

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