Rosai Dorfman disease or sinus histiocytosis with massive lymphadenopathy of the orbit
- R Khan1,
- P Moriarty1,
- S Kennedy2
- 1Department of Ophthalmology, Royal Victoria Eye and Ear Hospital, Dublin, Ireland
- 2National Ophthalmic Pathology Laboratory, Royal Victoria Eye and Ear Hospital, Dublin, Ireland
- Correspondence to: Dr Khan, Department of Ophthalmology, Royal Victoria Eye and Ear Hospital, Dublin, Ireland
- Accepted 9 September 2002
Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai Dorfman syndrome is a rare benign proliferative histiocytic disease of unknown origin. It predominantly affects the lymph nodes. The head and neck region usually in association with lymph node involvement, represents one of the most common extranodal areas affected by SHML. The other common extra nodal site is skin. Rarely, there is widespread dissemination with liver, kidney, respiratory organs, orbit, and eyeball involvement.1 The mean age of onset is 20 years (birth to 74 years).
Case report
A 57 year old woman with a 6 month history of double vision was referred to the Royal Victoria Eye and Ear Hospital, Dublin. She was found to have proptosis, ptosis, diplopia due to inferior rectus dysfunction, and restriction of elevation of the left eye. Her visual acuity was normal. Relevant investigations showed a high erythrocyte sedimentation rate (ESR) of 44 mm in the first hour, C reactive protein of 1.9 (normal less …
