Cystoid macular oedema associated with Rosai–Dorfman disease: a case report

BACKGROUND: The aim of this study is to report a case of cystoid macular oedema (CME) associated with Rosai–Dorfman Disease (RDD). CASE PRESENTATION: A 52-year-old male initially presented with a two-month history of a congested left eye. At presentation, visual acuity was 20/20 in the right eye and 20/80 in the left eye. Biomicroscopy showed conjunctival hyperaemia in the left eye with a slight elevation, suggesting a subconjunctival mass. Retinal fluorescein angiography and optical coherence tomography (OCT) revealed the presence of CME in the left eye. A clinical examination revealed nodular lesions in the ears and a lump in the subcutaneous tissue of the left arm. A biopsy of the subcutaneous lesion showed histological and immunohistochemical characteristics of RDD. The patient was treated with intravitreal triamcinolone (0.1 mL/4 mg). One month after treatment, there was complete regression of the oedema with a significant improvement in visual acuity to 20/20. CONCLUSIONS: This is the first reported case of RDD associated with cystoid macular oedema. Macular oedema responded to intravitreal treatment with triamcinolone.