DR. RAKHI P DCRUZ

Abstract

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy is a rare disorder-nonmalignant proliferation of distinctive histiocytes within lymph node & extranodal sites.

Case- 7 yr old male child who presented with gradual progressive painless nodular swelling B/Ly since 2 yrs.

O/E-complete ptosis RE, LE also showed similar rubbery swelling in the lower lid.Similar nodular masses-in the right cheek and genitalia. CT scan -isodense well-circumscribed lesion in the preseptal area extending to midorbit (RE)and upto posterior orbit LE.Debulking excision for RE was done and specimen sent for HPE AND IHC-confirmed RDD.Complete systemic work up along with BM biopsy,USG abdomen done.The child was given-systemic steroids[diffuse involvement to prevent recurrence]

CONCLUSION- RDD-suspected in young individuals with U/L orB/L slowly progressive proptosis with rubbery mass.Systemic evaluation-extranodal sites of involvement. A biopsy will confirm the diagnosis.