VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is an adult-onset autoinflammatory disorder characterized by systemic inflammation (vasculitis, arthritis, chondritis, dermatosis) and hematologic abnormalities (thrombosis, cytopenia, vacuolization of marrow precursors). The patient demonstrated some of the adult-onset inflammatory and hematologic features, in addition to recurrent eye pain, chemosis and orbital inflammation. This case describes an instance of VEXAS syndrome in a patient with uncommon orbital symptoms such as scleritis and myositis.

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