Purpose: We report an unusual case of orbital IgG4-related disease and discuss the distinguishing characteristics of the ophthalmic disease subtype.
Design: Case report.
Methods: Literature review and case description.
Results: Although lacrimal gland involvement has always been reported and elevated serum IgG4 is commonly observed, our case demonstrated neither in light of biopsy-proven IgG4 orbital involvement. A course of systemic steroids resolved our patient's periorbital abnormalities.
Conclusions: IgG4-related orbital disease mandates a high index of suspicion, and should be confirmed by tissue biopsy. Possible progression to MALT lymphoma necessitates close surveillance and may require repeat biopsy.