Lacrimal gland sparing IgG4-related disease in the orbit.

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.