Purpose: To report a unique case of isolated conjunctival inflammation from IgG4-related disease (IgG4-RD) confirmed by pathology.
Methods: A single interventional case of conjunctival IgG4-RD.
Results: A 63-year-old woman presented with a chronic, solitary, vascularized, tan-colored, and raised conjunctival lesion measuring 7.5 × 8.0 × 1.2 mm located at the temporal bulbar conjunctiva. An excisional biopsy was diagnostic of IgG4-RD based on the classic fibrosis pattern, 120 IgG4-positive plasma cells per high-power field, and an overwhelming majority of IgG4-positive cells among IgG plasma cells. No orbital or systemic involvement was found on clinical examination, imaging, and laboratory workup. The serum IgG4 level was normal (87.1 mg/dL). The patient was free of recurrence at 6-month follow-up.
Conclusions: Isolated conjunctival inflammation without orbital involvement can be a presentation of IgG4-RD.
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