Mikulicz syndrome is a chronic condition which is distinguished by the abnormal enlargement of glands in the head and neck, which includes:- 

  • Glands near the ears (parotids).
  • Glands around the eyes (lacrimal).
  • Glands of mouth (salivary). 
  • The tonsils and other glands in the soft tissue of the face and neck may also be involved. 

Causes

The exact cause of Mikulicz syndrome is not known. It is suspected to be an autoimmune disorder. 

This disorder is normally described as a benign disorder. Some scientists believe that Mikulicz syndrome should be considered a form of Sjögren syndrome.

The symptoms of Mikulicz syndrome may occur due to the excessive accumulation of certain white blood cells (lymphocytes) into many glands of the face, mouth, and/or neck.

Age and sex prevalence

Mikulicz syndrome affects more females than males and most often presents during the middle adult years.

Association 

Mikulicz syndrome always occurs in association with another underlying disorder such as:-

  • Tuberculosis.
  • Leukemia.
  • Syphilis.
  • Hodgkin’s disease
  • Lymphosarcoma.
  • Sjögren syndrome.
  • Lupus (SLE). 

People who have Mikulicz syndrome are at high risk for developing lymphomas.

Signs and Symptoms

  • Recurring fever. 
  • The fever may be accompanied by dry eyes, diminished tear production (lacrimation), and inflammation of various parts of the eyes (uveitis). 
  • Enlarged tear glands (lacrimal glands), leading to absent or decreased tears.
  • Sudden onset of extreme dryness in the mouth (xerostomia) that may lead to difficulty swallowing and tooth decay. 
  • Enlarged glands in the neck (parotid glands).
  • Hard, painless swellings of the salivary glands of the mouth and those near the ears (parotid). 
  • Glands near the jaw (submaxillary) may also become swollen. 

Symptoms may persist for long periods of time or come and go with frequent recurrences. 

Picture showing abnormal enlargement of glands in the head and neck.

Diagnosis

Biopsy of one of the swollen glands is essential to the diagnosis of Mikulicz syndrome. 

An ultrasound examination of the area may help to rule out other reasons for gland swelling. 

Management

Treatment is symptomatic, directed more toward the treatment of any underlying disease. 

Artificial tears may be used to maintain moisture in the eyes, and artificial saliva may be used to treat oral symptoms.

Soft moist diet. This may help to reduce the pain caused by chewing and swallowing. 

Other treatment is symptomatic and supportive.