No increase in IgG4-positive plasma cells in limbal rosai-dorfman disease

Saeed Alwadani, Shaun Robinson, Rory Myers, Esen K. Akpek, Charles G. Eberhart

Research output: Contribution to journalArticle

Abstract

PURPOSE:: Extranodal Rosai-Dorfman disease is a rare benign condition recently reported to sometimes show features of IgG4-related disease. The purpose of this study was to describe the corneal-limbal manifestation of the entity and to investigate whether numerous IgG4-positive plasma cells are associated with the disease at this site. METHODS:: This is an interventional retrospective small case series. RESULTS:: Two patients presenting with painless limbal mass lesions underwent total excisional biopsy with anterior lamellar keratoplasty for diagnostic and therapeutic purposes. Histopathologic evaluation of the specimens revealed inflammatory lesions containing atypical S100-immunoreactive histiocytes diagnostic of Rosai-Dorfman disease, but not an increase in the IgG4-positive plasma cells. Point mutations (V600E) in the BRAF oncogene were absent. CONCLUSIONS:: Rosai-Dorfmann disease should be considered in the differential diagnosis of limbal mass lesions. Involvement at this site was not associated with BRAF mutation or IgG4 abnormalities in the cases examined.

Original languageEnglish (US)
Pages (from-to)844-847
Number of pages4
JournalCornea
Volume33
Issue number8
DOIs
StatePublished - Aug 2014

Keywords

  • BRAF
  • IgG4
  • Rosai-Dorfmann disease
  • epibulbar
  • limbus

ASJC Scopus subject areas

  • Ophthalmology

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