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Diagnosis and Management of IgG4-Related Orbital Disease in a 54-Year-Old Man

This case study presents a 54-year-old man with a 5-year history of painless bilateral eyelid swelling, proptosis, and diplopia. The symptoms temporarily resolved with steroids but relapsed during tapering. A biopsy from the left infraorbital canal revealed lymphoplasmacytic infiltrate, fibrosis, and numerous IgG4-positive plasma cells. The serum IgG4 concentration was significantly elevated, confirming the diagnosis of IgG4-related orbital disease. This condition involves IgG4-positive cells and lymphocyte infiltration into various organs, emphasizing the need for careful management in affected patients.

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Diagnosis and Management of IgG4-Related Orbital Disease in a 54-Year-Old Man

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  1. TEACHING NEUROIMAGES NEUROLOGY RESIDENT AND FELLOW SECTION Deschamps et al. A 54-year-old man with eyelid swelling and diplopia.

  2. A 54-year-old man with a 5-year history of • painless bilateral eyelid swelling • proptosis • diplopia • Symptoms resolved with steroids but patient relapsed during tapering. Deschamps et al.

  3. Deschamps et al.

  4. IgG4-related orbital disease and enlargement of the trigeminal nerve branches • Biopsy from the left infraorbitalcanal: Lymphoplasmacytic infiltrate and fibrosis with numerous plasma cells stained positive for IgG4 (arrows). Meannumber of IgG4+ cells was 155 per high-power fields and ratio of IgG4+/IgG+ cells 89%. • Serum IgG4 concentration was 878 mg/dl (normal range 4-86 mg/dl). Deschamps et al. • IgG4-related disease is characterized by IgG4-positive cells and lymphocyte infiltration into various organs, including orbital tissues.

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