A case of orbital pseudotumor with a rare autoimmune disease that caused difficulty in diagnosis and treatment

A palpebral tumor or swelling is usually diagnosed as an angioma or malignant lymphoma. But we should consider a systemic disease for persistent swelling of the palpebrae. IgG4-related systemic disease has recently been characterized by elevated serum IgG4 concentrations and infiltration of many organs by plasmocytes expressing IgG4. The complications of IgG4-related systemic disease include autoimmune pancreatitis, tubulointerstitial nephritis, sclerosing cholangitis, inflammatory orbital pseudotumor, thyroiditis, and dacryoadenitis. A case of this rare condition of orbital pseudotumor is presented in which the swelling of the bilateral upper eyelids persisted in spite of repeated surgery previously performed in another hospital.