Bilateral anterior granulomatous uveitis associated with cutaneous Rosai-Dorfman disease

Background: Rosai-Dorfman disease (RDD) is a benign, rare, histiocytic disorder of unknown etiology. We describe the case of a 61-year-old woman who presented to the ophthalmology clinic with bilateral granulomatous anterior uveitis. Methods: The uveitis was controlled on topical prednisolone acetate 1%. One month after presentation, she developed generalized macules and papules, and dermatologic and systemic evaluations were performed. Results: On the basis of skin biopsy, purely cutaneous RDD was established. Conclusion: Anterior granulomatous uveitis may be the presenting sign of cutaneous RDD. Consultation is necessary for accurate diagnosis.