Inflammatory Pseudotumor of the Orbit: Inaugural Feature of Hyper 'igg4' Syndrome

Hyper IgG4 syndrome is a chronic condition characterized by fibro-inflammatory infiltration of the several organ systems. It leads to mass forming lesions and organ damage mimicking malignancy, inflammatory or infectious disorders. Hyper IgG4 syndrome is commonly misdiagnosed because of the lack of reliable biomarkers for this condition. We represent here a new case of a recurrent pseudotumor of the orbit revealing the diagnosis of hyper IgG4 syndrome. Relapses were managed by high dose corticosteroid therapy.