A novel clinicopathological entity of IgG4-related scle-rosing disease has recently been proposed that is characterized by infiltration of IgG4-positive plasma cells and T lymphocytes with fibrosis into various organs [1]. Mikulicz disease, characterized by symmetric bilateral swelling of the lacrimal and salivary glands, is considered a subtype of IgG4-related sclerosing disease [2], We report a case of concurrent extraocular muscle swelling and Mikulicz disease.A 68-year-old Japanese woman noticed swelling in her left eye in January 2005, and left palpebral edema developed in February 2008. She was referred to our hospital. She had a history of autoimmune pancreatitis (AIP).
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