Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient With Bilateral Lacrimal Gland Swelling

A 2-year-old male presented to ophthalmology following a short admission for dacryoadenitis, with a background of 6 months of bilateral fluctuating upper lid swelling, obstructive airway disease, and enlarged tonsils. He had been treated for an allergy-related disease with little effect and, post-hospital discharge, continued in a similar fashion as prior. Given the poor response to escalating allergy medication, a biopsy was taken from the right lacrimal gland. This demonstrated an eosinophil-rich small vessel vasculitis with extravascular eosinophilic granulomas, consistent with a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg–Strauss syndrome. The patient was placed on a tapering course of steroids and started on long-term mycophenolate immunosuppression. To our knowledge, this patient is the youngest case of EGPA with orbital involvement. The case and its heterogeneous presentation add to the growing knowledge of this rare condition.