CASE REPORT Unilateral Eyelid Mass as an Unusual Presentation of Ocular Adnexal IgG4-Related Inflammation Fabricio L. da Fonseca, MD,* Renata de Iracema Pulcheri Ramos, MD,* Patricia P. de Lima, MD,† Ariel B. Nogueira, MD,† and Suzana Matayoshi, MD, PhD* Purpose: IgG4-related sclerosing disease is a new clinicopatho- logic 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 lymphoplas- macytic 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. Key Words: IgG4, eyelid diseases, conjunctival disease, ocular adnexa (Cornea 2013;32:517–519) I gG4-related sclerosing disease is a recently described sys- temic autoimmune entity, characterized by specific clinico- pathologic findings such as abundant tissue IgG4-positive plasma cells and high serum IgG4 levels. 1,2 The disease was recognized as involving several organs, mainly the pan- creas and including retroperitoneal tissues, bile duct, gallblad- der, liver, salivary glands, lungs, and kidneys. 2 In recent studies, ocular adnexal disease has been described as a novel spectrum of disease affecting the lacrimal glands, orbital tissues, and other periocular structures. 3–6 However, in many cases, the presentation seems to be more diffuse, bilateral, and associated with lesions in other organs. 7 MATERIALS AND METHODS We present the case of a 67-year-old woman with left upper palpebral mass for 5 months. Her medical history was remarkable for diabetes mellitus. Initially suspected as infection, she was treated with 500 mg of cephalexin 4 times a day for 7 days, which resulted in clinical improve- ment, but with persistent redness and local infiltration (Fig. 1). A left upper palpebral biopsy was performed, and the specimen was diagnosed by means of the morphological features and immunophenotype (CD138, kappa, lambda, IgG, and IgG4). RESULTS Multislice abdominal computed tomography showed liposubstitution of the pancreas, but no abnormalities were detected on chest x-ray, magnetic resonance imaging scan of the hypophysis, autoimmune screen (anti-SSA, anti-SSB, antinuclear antibody, and rheumatoid factor), hematologic screen, or serum electrophoresis. The only abnormality was hyperglycemia at 180 mg/dL (reference range, 70–99 mg/dL) and a raised glycosylated hemo- globin level (7.8%; reference range, 4.1%–6.0%). Serum IgG level was 855 mg/dL (reference range, 952–1538 mg/dL) and serum IgG4 level 23 mg/dL (reference range, 8–140 mg/dL). The clinicopathologic study (Fig. 2) revealed lympho- plasmacytic and sclerosing inflammation with IgG4-positive plasma cells. There was no evidence of phlebitis or malig- nancy. Immunostaining identified numerous CD138/IgG/ IgG4-positive plasma cells (Figs. 2B–D) and a strong posi- tivity for kappa and lambda immunoglobulin chains (data not shown). The average number of IgG4-positive plasma cells in 10 high-power fields (·400) was 47.8 (with a range of 9–80 per high-power field). One high-power field covered an area of 0.19635 mm 2 . The degree of IgG4 infiltration in the tissue was scored as moderately positive for IgG4-related disease, with the mean number of IgG4-positive cells per high-power field between 30 and 99 cells. 5 The areas with the highest density of IgG4-positive cells were evaluated and an IgG4: IgG plasma cell ratio .40% was observed. 6,8 The patient was followed up monthly for 5 months, presenting spontaneous improvement of the lid mass. At the last visit, the lesion had reduced and the patient remained asymptomatic. Received for publication June 26, 2012; revision received September 25, 2012; accepted October 2, 2012. From the Departments of *Ophthalmology and †Pathology, University of São Paulo School of Medicine Hospital, São Paulo, Brazil. The authors state that they have no conflicts of interest to disclose. Reprints: Suzana Matayoshi, Department of Ophthalmology, University of São Paulo School of Medicine, Avenida Dr Enéas de Carvalho Aguiar, 255 6º Andar, Cerqueira César, CEP 05403-000, São Paulo, SP, Brazil (e-mail: suzana.matayoshi@gmail.com). Copyright © 2012 by Lippincott Williams & Wilkins Cornea  Volume 32, Number 4, April 2013 www.corneajrnl.com | 517