The tarsal plate manifestation of IgG4-related disease

Purpose: To describe a clinical case of bilateral biopsy-proven IgG4-related disease confined to the tarsal plate. Method: Interventional case report. Results: A 58-year-old woman presented with a tarsal swelling in the lateral part of the upper eyelids, with focal ulceration and mucus. Histology revealed fibrotic inflammation with increased IgG4-positive plasma cells fulfilling the criteria of IgG4-related disease (IgG4-RD). Serum IgG4 levels were increased, and pathological fluorodeoxyglucose uptake at positron emission tomography/CT scanning was restricted to the upper eyelids. After treatment with oral and topical prednisone, the tarsal lesions markedly regressed. Conclusions: Periorbital IgG4-RD may be confined to the tarsal plate. Treatment with systemic and topical steroids may induce significant regression.

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