Immunoglobulin G4-related chronic sclerosing dacryoadenitis

Abstract

A 47-year-old man had recurrent, episodic swelling of the right upper eyelid for about 2 years. There was a progressively enlarging, palpable mass over the superotemporal region of the right orbit without tenderness, local heat, or erythema. Ophthalmic examination revealed proptosis and ptosis of the right eye without vision decrease or ocular motility disturbance. An orbital computed tomography scan showed a huge lacrimal gland causing inferior displacement of the globe with proptosis. An anterior orbitotomy was done, and the pathological analysis revealed marked lymphoplasma cell infiltration with mild to moderate fibrosis surrounding the lobules of the lacrimal glands. No lymphoepithelial islands were seen. Immunohistochemically, about 40–50% of the immunoglobulin G (IgG)-positive cells were IgG4 positive, and the ratio of serum IgG4 to total IgG was 80.9%, compatible with a diagnosis of IgG4-related chronic sclerosing dacryoadenitis. IgG4-related chronic sclerosing dacryoadenitis may be a sign of Mikulicz disease, an IgG4-related systemic disease. Determination of the serum IgG4 concentration and IgG4/IgG ratio is necessary for clinical diagnosis. Long-term follow-up is also mandatory because of the possible complication of lymphoma.