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Research Letter
April 11, 2011

Rosai-Dorfman Disease Simulating Nodular Scleritis and Panuveitis

Author Affiliations

Author Affiliations: Department of Ophthalmology, Emory Eye Center, Atlanta, Georgia (Drs Payne, Wells, and Grossniklaus); and Department of Ophthalmology, Cole Eye Institute, Cleveland, Ohio (Dr Srivastava).

Arch Ophthalmol. 2011;129(4):512-526. doi:10.1001/archophthalmol.2011.56

Scleritis is a potentially sight-threatening inflammatory condition of the sclera that may be associated with keratitis, uveitis, glaucoma, and exudative retinal detachments.1 Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare histiocytic disorder characterized by massive, painless lymphadenopathy.2,3 While extranodal involvement is common, eye involvement is infrequent and most often seen in the orbit or eyelid.4 Direct ocular involvement is exceedingly rare.4 We report a case of Rosai-Dorfman disease that simulated nodular scleritis and panuveitis.

A 20-year-old woman with diabetes, hypertension, and hypothyroidism had a 3-day history of severe pain and vision loss in the left eye. She also noticed a slowly enlarging nodule on the left eye over the previous 5 months. Review of systems was negative for fever. Visual acuity was 20/20 OD and counting fingers OS. Intraocular pressure was 43 mm Hg OS. Examination showed an elevated perilimbal subconjunctival nodule, large keratic precipitates, 3+ anterior segment inflammation, 2+ vitreous haze, and subretinal exudate (Figure 1A and B). Ultrasonography revealed diffuse scleral thickening. Laboratory testing revealed no leukocytosis, a normal angiotensin-converting enzyme level, a nonreactive rapid plasma reagin test result, and a slightly elevated erythrocyte sedimentation rate (25 mm/h). Tuberculosis skin testing and chest radiography results were normal. Results of testing for antinuclear antibodies, anticytoplasmic nuclear antibodies, and HLA-B27 were negative.