Open-access A rare case of Rosai-Dorfman disease involving the lacrimal sac

A 27-year-old patient presented with seropurulent ocular discharge and prop-tosis. Orbital computed tomography revealed a right intraorbital mass (blue arrow) abutting the medial rectus muscle (Figure 1A) and bilateral dacryocystoceles (green arrows; Figure 1B). Histopathological examination of a lacrimal sac biopsy demonstrated histiocytic proliferation (*) with emperipolesis (purple arrow; Figure 1C). Immunohistochemical analysis showed positive staining for S100 and CD68 and negative staining for CD3, CD20, and CD1a (Figure 1D), confirming the diagnosis of Rosai-Dorfman disease (RDD), a rare non-Langerhans cell histiocytic disorder. Ocular involvement occurs in approximately 7% of patients with RDD(1-3).

  • Funding:
    This study received no specific financial support.

Data Availability Statement:

The datasets generated and/or analyzed during the current study are already available.

REFERENCES

  • 1 Ma YL, Liang ZP, Xu SE, Yang ZH, Peng Y, Sun XQ, et al. Rosai-Dorfman disease (RDD) in the paraglottic space: report of a case and review of literature. Int J Clin Exp Pathol. 2015;8(10):13532-13538.
  • 2 Barbalho CEC, Vasconcelos DN, Ximenes Filho JA, Araripe AA, Ferreira FV de A. Rosai-Dorfman Disease as a differential diagnosis in vocal cord ulceration. Braz J Otorhinolaryngol. 2010;76(6):795.
  • 3 La Barge DV, Salzman KL, Harnsberger HR, Ginsberg LE, Hamilton BE, Wiggins RH, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol. 2008;191(6):W299-306.

Edited by

  • Editor-in-Chief:
    Newton Kara-Júnior
  • Associate Editor:
    Carlos Augusto Moreira Neto

Publication Dates

  • Publication in this collection
    28 Aug 2026
  • Date of issue
    2026

History

  • Received
    27 Apr 2026
  • Accepted
    23 June 2026
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