Unilateral eyelid mass as an unusual presentation of ocular adnexal IgG4-related inflammation

IgG4-related sclerosing disease is a new clinicopathologic systemic entity, which can involve ocular adnexal structures, such as lacrimal glands and orbital tissues. However, this entity seems to display a more diffuse, frequently bilateral, pattern in such cases. Conjunctival involvement has not been reported in this disease.

We report the case of a 67-year-old woman with left upper palpebral mass for 5 months. A biopsy was performed, and the patient underwent systemic evaluation for IgG4-related disease.

The clinicopathologic study revealed intense lymphoplasmacytic and sclerosing inflammation, with IgG4-positive plasma cells and an IgG4:IgG ratio >40%. The conjunctiva was also involved, which was different from previous studies. The only abnormality was hyperglycemia at 180 mg/dL (reference range, 70-99 mg/dL) and a raised glycosylated hemoglobin at 7.8%. Serum IgG and IgG4 levels were normal.

Our case presented an unusual framework of ocular adnexal IgG4-related inflammation, with a more benign and restricted palpebral manifestation involving the conjunctiva.