A novel concept of Mikulicz's disease as IgG4-related disease

Portrait of Mikulicz-Radecki (1850−1905) [cited from reference 7].

He contributed immensely to cancer surgery. Besides the development of operations on digestive organs, he contributed to the area of head and neck surgery. Mikulicz forceps, still the most widely used peritoneal forceps, is one of the surgical instruments devised by Mikulicz.

Illustration of the first reported case of Mikulicz's disease (MD). A 42-year-old male developed bilateral swelling of the lacrimal, parotid, and submandibular glands.

Facial views and computed tomography images of MD patients. The patients show persistent symmetrical enlargement of the lacrimal (arrow heads) and submandibular glands (arrows).

Comparison of serum levels of IgG subclasses (a) and ratios of each IgG subclass/total IgG (b) between MD and SS. The statistical significance was determined by the Mann–Whitney U test. Serum IgG4 levels were significantly higher in MD than in SS for levels (a) as well as ratios (b). *P values of <0.005 compared to the IgG4 level of SS.

Immunohistochemistry for IgG4 in salivary glands in MD (a) and SS (b). Immunoperoxidase stain, hematoxylin counterstain, ×200. Abundant IgG4-postive cells infiltrate around acinar and ductal cells in the submandibular glands of MD patients (a), whereas no infiltrating IgG4-positive cells are observed in the labial salivary glands of SS patients (b).

Changes in salivary secretion by Saxon's test after steroid treatment. Administration of glucocorticoids for 2 months increases salivary secretion from 1.95g/2min to 3.55g/2min. *P values of <0.01 compared to pre-treatment salivary secretion (P<0.01).