Rosai-Dorfman disease (RDD) is a rare idiopathic disorder affecting predominantly young adults.1 A bilateral massive painless enlargement of lymph nodes, predominantly in the cervical area, characterizes the disease. Additionally, several ophthalmic manifestations involving the eyelid, orbit, and lacrimal gland have been reported.2,3 The soft tissue surrounding the eye is infiltrated by a circumscribed rubbery, nontender mass, leading to variety of clinical signs and symptoms, eg, exophthalmos, blepharoptosis, conjunctivitis, keratitis, diplopia, dry eyes, and photophobia. The involvement ofintraocular structures is less frequently described.4,5 We report a case of RDD with bilateral serous retinal detachments and its resolution after pharmacologic treatment.
A 60-year-old man with bilateral enlarged inguinal lymph nodes underwent a biopsy and was diagnosed as having RDD (Figure 1B). Two years later, the patient noticed a decrease in his visual acuity. His visual acuity was 20/40 OD and 20/50 OS. On slitlamp examination, anterior uveitis with cells in the anterior chamber was seen. Biomicroscopy demonstrated mild macular edema and some focal hyperpigmentations in the midperiphery of both fundi. Although the patient was treated with topical corticosteroids, his visual acuity decreased to 20/100 OU during the next 6 months. During this period, the patient developed massive bilateral serous retinal detachments with shifting fluids in the inferior quadrant; these detachments remained during the next 2 years.B-scan ultrasound demonstrated a retinal detachment with underlying sonolucent choroidal thickening (Figure 2A). Because of the long-term retinal detachment, we considered antimetabolic therapy, and treated the patient daily with 50 mg of prednisolone orally and 3 times per day with 2% cyclosporine eye drops for 10 weeks. During this time, thepatient's visual acuity increased to 20/50 OU. The choroidal thickening remained constant; however, the retina reattached completely, as seen on B-scan ultrasound (Figure 2B). Optical coherence tomography (OCT) showed a reattached retina with circumscribed hyperreflective areas in the retina and choroid (Figure 1A). Although the therapy was gradually stopped during the next 3 months, the anatomical and functional status remained stable.
A, Sagittal optical coherence tomographic image of the temporal macula of the right eye. The inner retina has a hyperreflective orange to reddish color, consistent with circumscribed infiltrates (asterisks). The photoreceptors represent a hyporeflective area of black to blue colors. The second hyperreflective band represents the retinal pigment epithelium–choriocapillaris complex (arrow). The underlying choroid has an unusually high reflectivity in yellow to greenish colors. B, Immunohistochemical staining of paraffin sections of lymph node specimens. The diagnosis of Rosai-Dorfman disease is histologically based on lymphophagocytosis. The histiocytes express strong positivity with antibodies against S100 protein in the cytoplasm and nuclei (arrows).
Sagittal B-scan ultrasound of the inferior quadrant of the right eye. A, Prior to treatment, the retina was detached and the choroid appeared as a low reflective band approximately 2 mm thick. B, Ten weeks after treatment, the retina was reattached. The choroidal thickening remained constant, although the tissue became less echolucent.
Intraocular manifestations with uveitis are rare in patients with RDD. Foucar et al2 reported the microscopic findings from 13 enucleated eyes with RDD, and only 1 eye had extensive infiltration of predominant histiocytes in the entire uvea. Histologic examination of the eye demonstrated retinal detachment over approximately 1 mm of thickened choroid and a focal infiltration of histiocytes in the retina. We believe that our patient has the same intraocular condition.
The rare nature of the disease and the self-limiting prognosis reduces the knowledge about effective treatment. Treatment with only topical corticosteroids is not effective in reducing anterior uveitis.2 However, the retinal detachment in our patient responded quickly to treatment combining topical cyclosporine and systemic corticosteroids.
Our patient developed rare, vision-threatening intraocular infiltrates. These lesions led to back scattering of laser light during OCT and possibly to abnormally high signals on the false color image. The OCT image morphologically assessed the anatomy in vivo and confirmed intraretinal and choroidal infiltrates in a patient with RDD.
The authors have no relevant financial interest in this article.
Juan Rosai, MD, New York, NY, reviewed the subsequent cervical lymph node biopsy and confirmed the histopathologic diagnosis in this case.
Corresponding author: Carsten H. Meyer, MD, Department of Ophthalmology, Philipps University–Marburg, Robert-Koch-Strasse 4, 35037 Marburg, Germany(e-mail: firstname.lastname@example.org).
Meyer CH, Sel S, Hörle S, Burg J, Kroll P. Rosai-Dorfman Disease With Bilateral Serous Retinal Detachment. Arch Ophthalmol. 2003;121(5):733-735. doi:10.1001/archopht.121.5.733