A 48-year-old male presented with intermittent bilateral eyelid swelling for 5 years and progressively enlarging well-demarcated yellow eyelid lesions. On exam, patient had chemosis, and reduced up gaze in the right eye, prompting orbital imaging with
MRI which demonstrated enlargement of the rectus muscles and bilateral lacrimal glands. Patient underwent bilateral upper lid blepharoplasty with orbitotomy for biopsy of the lacrimal gland. Histopathology of the lesions demonstrated numerous foamy
macrophages, touton giant cells, characteristic findings of xanthogranulomatous disease. In addition, there was significant IgG4 positivity. Patient was started on a course of PO steroids for the orbital involvement; however patient is lost to follow
up since surgery and subsequent systemic work-up including serum IgG4 levels has not been completed.
Presentation Date: 10/01/2020
Issue Date: 02/12/2021