Orbital, adnexal, and unusual systemic involvement in Rosai-Dorfman disease.

PURPOSE: To describe the unusual clinical course of a patient with Rosai-Dorfman disease (RDD) affecting the eyelid and orbital tissues and involving the spinal cord.

METHODS

Case report.

RESULTS

A 68-year-old Indian man first presented in 1994 with a right lower eyelid lump for 1 year. An en bloc excisional biopsy was reported to show "reactive lymphoid hyperplasia with sclerosis." The patient subsequently defaulted follow-up and presented again in 1999 with bilateral lower eyelid masses and proptosis. Computerized tomography showed bilateral orbital, ethmoidal sinus, and frontal sinus soft tissue masses. Bilateral excisional biopsies of the orbital and eyelid masses showed histologic features of RDD. The patient had a history of paraplegia with decompression laminectomy and excision of an epidural mass in 1994. In addition, he underwent excision of soft tissue masses from the abdominal wall in 1993. Retrospective review of the histologic specimens from these two areas showed a histologic picture similar to that of eyelid specimens (in 1994 and 1999).

CONCLUSIONS

It is important to consider RDD in addition to lymphoproliferative disorders in a patient with orbital and ocular adnexal masses. The initial histologic presentation may not be pathognomonic.