Unilateral Eyelid Mass as an Unusual Presentation of Ocular Adnexal IgG4-Related Inflammation

Abstract

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

IgG4-related Disease Masquerading as Recurrent Scleritis and Chronic Conjunctivitis

“…10 Only 1 case of IgG4-RD involving the sclera 11 and 2 cases of conjunctival location of the disease have been reported: both conjunctival locations were associated with typical orbital inflammation 12 and eyelid lesions. 13 In our cases, atypical ophthalmic manifestations were the only manifestations of the disease. There were no signs of systemic or lacrymal involvement.…”

Isolated Conjunctival Inflammation Suggestive of IgG4-Related Disease

“…Recent case reports described conjunctival involvement in patients with IgG4-related disease [6,7,8]. Here, we describe an isolated inflammatory conjunctival lesion without orbital involvement that was suggestive of IgG4-related disease.…”

“…While the lacrimal gland is the most common ophthalmic manifestation in IgG4-related disease, the conjunctiva was initially believed to be spared [4]. Recent case reports have shown conjunctival involvement in IgG4-related disease with adnexal involvement [6,8] or in isolation [7]. …”

Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit

“…Neither Sato et al nor this study had patients with IgG4-ROD originating from conjunctival or subconjunctival tissue. However, prior research suggested that IgG4-ROD can also develop at conjunctival tissue [23,24].…”