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Primary Skeletal Rosai Dorfman Disease of Distal Tibia

Arun VP

Rosai Dorfman disease is a non-langerhan cell histiocytosis mainly manifesting as massive painless cervical lymphadenopathy associated with constitutional symptoms in young adults. Isolated skeletal involvement by Rosai Dorfman disease is extremely rare. 48-year-old lady presented with pain and swelling of left ankle of six month’s duration. Radiological investigations revealed lytic lesion of Distal Tibia with cortical breach and extraosseous extension. Histopathological examination of the excised lesion clinched the diagnosis. This presentation of the disease is very uncommon as it primarily involves the osseous tissue without the involvement of lymph nodes, and, considering the age of the patient, middle aged individual. A broader array of differential diagnosis should be kept in the mind of a clinician over and above a neoplastic or infective pathology.