A Case of Orbital Rosai-Dorfman Disease with IgG4 Positive Plasma Cells. (22nd July 2013)

• Record Type: Journal Article
• Authors: Mudhar, Hardeep Singh; Duke, Roseline
• Is Part Of: Orbit. Volume 32:Number 5(2013)
• Page Start: 315
• Page End: 317
• Publication Date: 2013-07-22
• Subjects: IgG4 -- orbit -- Rosai-Dorfman; Eye-sockets -- Surgery -- Periodicals; Eye-sockets -- Diseases -- Periodicals; Orbit -- surgery -- Periodicals; Orbital Diseases -- Periodicals; 617.7
• DOI: 10.3109/01676830.2013.799705
• Languages: English
• ISSNs: 0167-6830

Abstract

Abstract: A case of a 9-year-old Black African male with right-sided proptosis is presented. The clinical interpretation was that of orbital rhabdomyosarcoma and an incisional biopsy was performed. This revealed the typical features of Rosai-Dorfman disease, featuring S100 positive non-Langerhans histiocytes with emperipolesis. There were also numerous plasma cells in the biopsy, in the more fibrotic areas. More than 40% of these plasma cells were positive for IgG4. This case represents the first case in the orbit to make the association of IgG4 plasma cell positivity with Rosai-Dorfman disease. Potential associations are discussed, and the relevance of this finding to ophthalmic pathology interpretation and differential diagnosis are highighted.