More Than Meets the Eye: IgG4-related Disease presentation as fluctuating ocular symptoms

Abstract

A 13-year-old boy with seasonal allergies and purported mild asthma originally presented with a month-long history of bilateral upper eyelid swelling and pruritis to the emergency department. He had intermittent relief of symptoms on antihistamines. He was diagnosed with dacryocystitis and discharged on Bactrim and prednisone. Over the next month his eye swelling worsened, localizing to his right eye and developing pain despite extending antibiotics. He was then evaluated in hospital with a presumptive diagnosis of pre-septal cellulitis and admitted for IV antibiotics. He was discharged on Clindamycin and a referral to allergy/immunology was made. He continued to have poor response to outpatient treatment by Ophthalmology for presumed infectious dacryocystitis. Eventually, he was admitted for an excisional biopsy of his right lower lid and probing of his lacrimal system. Fibrotic tissue with some pus drainage was found at the time, cultures being eventually negative.The patient was evaluated by Allergy & Immunology where his symptoms did not fit a classic angioedema. Labs were obtained to rule out nephrotic syndrome and IgG4-related disease. Interestingly, patient’s IgG4 subclass was significantly elevated, and IHC staining was added on to the biopsy done with ophthalmology previous which supported the diagnosis of IgG4-related disease.IgG4- related disease is an immune-mediated fibroinflammatory disease with varied clinical presentation. Our case adds to the literature of IgG4-related disease with a curious case of a pediatric male patient with bilateral eye lid swelling which was wrongly treated as dacrocystitis. In conclusion, IgG4-related disease should be in the differential diagnosis of pediatric patients presenting with atypical eye swelling not fitting into angioedema.