Mikulicz syndrome K11.81

Rare, chronic, IgG4-associated (autoimmune) disease of the lacrimal and salivary glands (see below IgG4-associated diseases), which can be monoorganic, but also accompanying in various general and systemic diseases, e.g. Hodgkin's and non-Hodgkin's lymphomas, leukemias and sarcoidosis; also occasionally in tuberculosis, syphilis, sialosis and hyperthyroidism.

The syndrome seems to occur preferentially in the Asian population.

High serum levels of IgG4; no detection of SS-A or SS-B antibody (DD Sjögren's syndrome)

Clinic, Sonography

Sjögren's syndrome (detection of SS-A or SS-B antibodies).

Good effectiveness of glucocorticosteroids. Treatment of a possible underlying disease.

Probably the first description of an IgG4-associated disease in the salivary gland comes from Mikulicz-Radecki . Later work identified the mononuclear infiltrate in the so-called Mikulicz syndrome (K11.8) as an image characterized by IgG4-positive plasma cells.

1. Awan KJ (1976) Mikulicz disease and Mikulicz syndrome. South Afr Med J 69: 458
2. Azzopardi YES, Evans DJ (1971) Malignant lymphoma of the parotid associated with Mikulicz disease (benign lymphoepithelial lesion). J Clin Pathol 24: 744.
3. Kowalczyk J (2003) Jan Mikulicz-Radecki (1850-1905)--father of surgical endoscopy. S Afr Med J 93: 509-510
4. Moriyama M et al (2013) Clinical characteristics of Mikulicz's disease as an IgG4-related disease. Clin Oral Investig 17:1995-2002.
5. Penfold CN (1985) Mikulicz syndrome. J Oral Maxillofac Surgery 43: 900
6. by Mikulicz-Radecki (1892) On a peculiar symmetrical disease of the tear and oral salivary glands. In: Contributions on surgery. Commemorative publication dedicated to Theodor Billroth, Stuttgart,pp. 610-630

7. Shimizu M et al (2015) Effectiveness of imaging modalities for screening IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) and for differentiating it from Sjögren's syndrome (SS), with an emphasis on sonography. Arthritis Res Ther 17: 223.