IgG4-related ocular surface disease: A clinicopathological diagnosis

A 56-year-old woman presented with chronic ocular surface inflammation and symblepharon. Histopathological examination revealed a dense lymphoplasmacytic infiltrate with storiform-like fibrosis. Immunohistochemistry demonstrated numerous CD138-positive plasma cells and IgG-positive staining and increased IgG4:IgG ratio, findings consistent with an IgG4-related fibro-inflammatory process. Characteristic features of IgG4-related disease include plasma cell-rich infiltrates, storiform-like fibrosis, and an increased IgG4:IgG ratio [Fig. 1a-f].[1–5] While lacrimal gland and orbital involvement are common, ocular surface fibrosis is rare and underreported.[2,4] Recognition of plasma cell markers such as CD138 is essential in differentiating IgG4-related disease from other chronic inflammatory disorders as an adjuct marker.[4] Early recognition allows timely immunomodulatory therapy to prevent irreversible ocular surface damage and vision loss.[4]Figure 1: Panel a: Active ocular surface inflammation with adhesion to corneal surface. Panel b: H and E stain revealing dense lymphoplasmacytic infiltrate with storiform fibrosis. (X-200). Panel c: Immunostaining demonstrating clusters of CD138+ plasma cells.(X-200). Panel d: Progressive ocular surface inflammation during course of treatment. Panel e: Immunohistochemistry highlighting dense plasma cell infiltrates. (X-200). Panel f: IgG4 immunostaining showing elevated IgG4-positive plasma cells. (X-200)Ethics/Consent statement Written informed consent was obtained from the patient for publication of clinical details and associated images. Authors’ contributions Dr. Parul Jain conceptualized the case report, supervised clinical management, and critically revised the manuscript. Dr. Sumeet Singla contributed to clinical data collection and manuscript drafting. Dr. Ravindra Saran, Dr. Kapil Chalana and Dr. Rishita Rao contributed to pathological evaluation and interpretation of histopathology findings. Dr. Sunidhi Rauthan assisted in data collection, literature review, and manuscript preparation. All authors reviewed and approved the final manuscript. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.