Bilateral anterior granulomatous uveitis associated with cutaneous Rosai-Dorfman disease

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.

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.

On the basis of skin biopsy, purely cutaneous RDD was established.

Anterior granulomatous uveitis may be the presenting sign of cutaneous RDD. Consultation is necessary for accurate diagnosis.