Two Cases of IgG4-Related Sclerosing Disease Presenting at the Tarsal Conjunctiva

Abstract

Purpose: IgG4-related disease is a relatively recent disease entity, increasingly recognized for its involvement of the orbital tissues and lacrimal glands. We report a series of 2 patients with unusual presentations of IgG4-related sclerosing disease of the eyelid. Methods: We report of 2 unusual cases of eyelid conjunctival mass where biopsy result was found to be IgG4-related sclerosing disease. Results: A 66-year-old man with a 9-month history of right eye discomfort was incidentally found to have large and firm conjunctival nodules over the right upper eyelid tarsal plate; the largest size was 13 x 8 mm. An incisional biopsy showed IgG4-related sclerosing disease with IgG4 plasma cells over 100 HPF and IgG4:IgG ratio over 80%. The patient had normal serum IgG level. Topical antibiotic and steroid ointment was given afterward. Residual nodules resolved spontaneously. The second case was a 51-year-old female with a 2-month history of right eye epiphora. An excisional biopsy showed increased proportion of IgG4+ plasma cells. The IgG4/IgG plasma cell ratio was 70%. Presence of lacrimal glands was also noted in the specimen. Serum IgG level was normal and IgG4 was high at 1.228 g/L (N = 0.168-1.000 g/L). In both cases computed tomography (CT) of the orbit showed no orbital mass and there was no systemic involvement. Both patients did not receive any systemic steroid treatment. There was no recurrence after 3 years and 7 months in the first case and 15 months in the second case. Conclusions: IgG4-related disease can have a wide range of ocular manifestations including eyelid conjunctival involvement.