Rosai-Dorfman Disease Simulating Nodular Scleritis and Panuveitis

Extranodal Rosai-Dorfman of the Pancreas Presents with Bilateral Granulomatous Anterior Uveitis

“…Treatment usually entails topical and oral steroids, but radiation, chemotherapy, and surgical excision is used in cases with severe systemic disease. Poor visual outcomes in the literature have been associated with the development of complications including uveitic glaucoma, cystoid macular edema with serous retinal detachment, and panuveitis [4, 7, 8]. …”

“…Close histological examination is important to differentiate RDD from other lymphocytic diseases in cluding histiocytosis X, histiocytic lymphoma, orbital pseudotumor, Hodgkin’s lymphoma, and monocytic leukemia. Epibulbar lesions in RDD can mimic both lymphoma and scleritis [4, 9, 10], necessitating biopsy if the lesion did not improve with treatment. Although the etiology is unknown, RDD is thought to be a reactive process and has been associated with Epstein-Barr virus [10], varicella zoster [11], human immunodeficiency virus [12], and other infectious etiologies such as tuberculosis [7, 13].…”

Sclerouveitis Associated with Rosai-Dorfman Disease in an HIV-infected Patient

“…Uveitis has been reported in a few cases, including 2 cases of anterior uveitis in children 5,6 and 3 cases with posterior segment involvement. 2,7,8 Several cases of epibulbar masses, with and without concomitant uveitis, have been reported. [7][8][9] These cases may mimic scleritis, but no cases of true scleritis associated with this disease appear in the literature.…”

“…2,7,8 Several cases of epibulbar masses, with and without concomitant uveitis, have been reported. [7][8][9] These cases may mimic scleritis, but no cases of true scleritis associated with this disease appear in the literature. We believe our patient's presentation to be unique, in that he had no evidence of a discrete epibulbar mass, and instead presented with a more diffuse, bilateral anterior scleritis and associated nongranulomatous anterior chamber inflammation.…”

“…Surgical resection may be necessary if a discrete mass is present, 2 and patients with uveal involvement have been treated with varying combinations of topical and oral corticosteroids, as well as systemic immunosuppressive therapy. 7,8 Four previous cases of Rosai-Dorfman disease in HIV-infected patients have been reported. In these cases, CD4 count at initial presentation ranged from 204 to 350 cells/mm 3 , 10,11 a range similar to that of our patient.…”