Beyond the usual suspects: Rosai–Dorfman disease as a cause of sclerokeratouveitis with epibulbar nodules

A 38-year-old male presented with bilateral ocular redness and vision decline over 4 months. Visual acuity was 6/6P in right eye and 6/36 in the left eye. The right eye exhibited congestion, a nasal epibulbar nodule, keratic precipitates (KPs), anterior chamber (AC) cells 1+, and a normal fundus. The left eye demonstrated multiple epibulbar masses, superficial punctate keratitis, fine to medium-sized KPs, AC cells and flare 2+, grade 1 vitritis, and a normal retina. Initial investigations, including an incisional biopsy, were inconclusive, except for a positive Mantoux test. Initial treatment with empirical antitubercular therapy and systemic steroids resulted in inadequate improvement. The second excisional biopsy was marked by histiocytes and classical emperipolesis, confirming extranodal Rosai–Dorfman disease (RDD). Surgical excision, systemic methotrexate (20 mg/week), and steroids significantly improved symptoms over 5 months. This unique presentation of sclerokeratouveitis, without the typical lymphadenopathy associated with RDD, emphasizes the necessity for high clinical suspicion and multidisciplinary management in such cases.