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

A 27-year-old patient presented with seropurulent ocular discharge and proptosis. 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).

REFERENCES

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Submitted for publication: April 27, 2026.
Accepted for publication: June 23, 2026.

Data Availability Statement: The datasets generated and/or analyzed during the current study are already available.

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

Funding: This study received no specific financial support.

Disclosure of potential conflicts of interest: The authors declare no potential conflicts of interest.