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Mikulicz syndrome

Monday 5 November 2007

Mikulicz disease, Miculicz syndrome. Ent. Mikulicz, 1892.

Definition: Mikulicz syndrome is a clinical chronic condition characterized by the abnormal enlargement of glands in the head and neck, including those near the ears (parotids) and those around the eyes (lacrimal) and mouth (salivary). The tonsils and other glands in the soft tissue of the face and neck may also be involved.

Mickulicz’s syndrome is a general term applied to concomitant enlargement of the lacrimal and major salivary glands which may occur as a result of a wide variety of disorders including Sjogren disease , sarcoidosis , and lymphomas .

Although the disorder is almost always described as benign, it always occurs in association with another underlying disorder such as tuberculosis, leukemia, syphilis, Hodgkin lymphomas, lymphomas, Sjogren syndrome, or systemic lupus erythematosus (SLE).

People who have Mikulicz syndrome are at heightened risk for developing lymphomas. Some people with Mikulicz syndrome may experience recurring fevers. The fever may be accompanied by dry eyes, diminished tear production (lacrimation), and inflammation of various parts of the eyes (uveitis). Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs.

The exact cause of Mikulicz syndrome is not known. Some scientists believe that Mikulicz syndrome should be considered a form of Sjogren syndrome.

Etiology (Examples)

- salivary benign lymphoepithelial lesion
- sarcoidosis
- leukemias
- lymphomas
- lymphoepithelial sialadenitis (myoepithelial sialadenitis )