Optic Nerve Involvement with Panuveitis in Sweet Syndrome

Purpose: To report a case of a patient with optic nerve involvement in neuro-Sweet syndrome and review the literature on this systemic disease.

Methods: Interventional case report and review of the literature.

Results: A 49-year-old man developed an acute exacerbation of bilateral panuveitis and optic disc edema together with symptoms of a papular rash on his forearms, fevers, malaise, and arthalgias. Laboratory findings revealed an aseptic meningitis and neutrophilic leukocytosis. Biopsy of the skin rash demonstrated an intense neutrophilic infiltrate in the dermis consistent with the diagnosis of neuro-Sweet syndrome. The patient continued to require corticosteroids for inflammation control in spite of immunomodulatory therapies, including tumor necrosis factor-alpha inhibitor infliximab.

Conclusions: Sweet syndrome can present with optic nerve involvement. Screening for underlying etiologies, including autoimmune disease, malignancy, and offending drugs, is important for targeted therapy.