Rosai Dorfman Disease - A Rare Presentation of Cervical Lymphadenopathy - A Case Report

Rosai Dorfman disease is an uncommon histiocytic disorder presenting as bilateral cervical lymphadenopathy in children and young adults. Under the revised classification of histiocytic disorders, it is classified in the` R’ group of histiocytosis. Cutaneous Rosai -Dorfman disease is regarded as a separate entity which falls under the`C’ group of histiocytosis according to this classification system. We herein report a case of cervical lymphadenopathy who initially developed swelling in the right side of the neck and later developed in the left side of the neck. Patient underwent mantoux test which showed induration of 22mm which was significant. No other symptoms like cough with expectoration, loss of appetite was present.chest x ray was normal. Tuberculous Lymphadenitis was suspected. FNAC was done but confirmatory diagnosis was not obtained, so Excision biopsy was done. Finally, microscopic examination revealed marked emperipolesis with sheets of mature lymphocytes and plasma cells which is pathognomic cytoarchitecture of Rosai Dorfman disease. No evidence of granuloma/ RS cells/ atypia seen. Immunohistochemistry showed S -100 – strong positivity