IgG4-related Disease of the Head and Neck

IgG4-related disease is an uncommon sclerosing and inflammatory mass-forming disease that may affect a single organ or be systemic. The prototypical example of the disease is type 1 autoimmune pancreatitis. After the pancreatobiliary system, the head and neck is the next most common site for involvement by IgG4-related disease. Here, we describe the clinicopathologic features of the head and neck involvement by this disease process with particular attention to involvement of the major salivary glands, the lacrimal glands and periorbital tissues, the upper aerodigestive tract, the thyroid gland, lymph nodes, the ear, and the skin and soft tissues.