Abstract

<h3>Introduction</h3> We present a case of eyelid edema initially thought to be due to allergic conjunctivitis, angioedema and periorbital cellulitis that was eventually discovered to be due to Immunoglobulin G4-related disease (IgG4-RD). <h3>Case Description</h3> Our patient was a 57 year-old female with asthma and allergic rhinosinusitis, who presented to clinic with right eyelid edema, injected conjunctiva, and pruritus. She had a history of left eyelid swelling attributed to meibomian gland dysfunction. Initially we treated her for allergic conjunctivitis. A year later, she was treated with clindamycin for periorbital cellulitis, and the year after that, treated for angioedema by the emergency department. Soon after, she developed left eyelid swelling and thick eye discharge. Neuro-ophthalmology evaluation was concerning for polyclonal lymphoid proliferation and sclerosing orbital pseudotumor. Serum IgG4 was elevated and a lacrimal gland biopsy revealed reactive lymphoid hyperplasia compatible with IgG4-RD. She was treated with prednisone for several weeks with good response. <h3>Discussion</h3> IgG4-RD is an uncommon medical condition that can present similarly to more common diseases and can be challenging to diagnose.

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