Clinical course of von Szily reaction: Case report and comprehensive review of the literature

PURPOSE: To describe a rare case of von Szily reaction (VSR) accompanied by a comprehensive review of the literature. OBSERVATIONS: A 57-year-old woman with herpes zoster ophthalmicus (HZO) associated with ipsilateral sectoral scleritis and anterior uveitis (sclerouveitis) subsequently developed contralateral necrotizing retinitis, leading to a diagnosis of VSR. A literature review revealed 10 additional cases of VSR. The full VSR cohort of 11 subjects included six women and five men, had a median age of 39 years (range 21–78 years), and most presented with HZO (n = 7, 63.6%), often associated with either ipsilateral anterior uveitis (n = 5; 45.5%) or keratitis (n = 4; 36.4%). All 11 cases developed necrotizing retinitis in the fellow eye, at a median of six weeks following onset in the sentinel eye. The most frequently implicated agent was varicella zoster virus (VZV; n = 8, 72.7%). A high proportion of the eight patients with VZV-associated VSR were identified as having increased risk of VZV reactivation, including age of 50 years or greater (n = 5, 62.5%), an underlying malignancy (n = 3, 37.5%), and/or use of immunosuppressive medication (n = 2, 25.0%). CONCLUSION: This was the first reported case of VSR presenting as HZO-associated with sclerouveitis. A comprehensive literature review revealed that most previously reported cases presented with HZO associated with isolated anterior uveitis and/or keratitis, and that all reported cases of VSR developed necrotizing retinitis in the fellow eye, typically within two months of initial presentation. Patients with VZV-associated VSR often had known risk factors for VZV reactivation.