Mikulicz syndrome is a 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. Although the disorder is almost always described as benign, it always occurs in association with another underlying disorder such as tuberculosis, leukemia, syphilis, Hodgkin's disease, lymphosarcoma, Sjogren syndrome, or lupus (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. .
Mikulicz syndrome is characterized by the sudden onset of extreme dryness in the mouth (xerostomia) that may lead to difficulty swallowing and tooth decay. Other symptoms include enlarged tear glands (lacrimal glands), leading to absent or decreased tears; enlarged glands in the neck (parotid glands); hard, painless swellings (tumefactions) of the saliva glands (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. The symptoms of Mikulicz syndrome are very similar to those of Sjögren syndrome and some researchers suspect that they may be the same disorder (see Related Disorders section of this report). .
The exact cause of Mikulicz syndrome is not known, although it is suspected to be an autoimmune disorder. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.
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. .
Mikulicz syndrome affects more females than males and most often presents during the middle adult years.
It often occurs in combination with Sjögren syndrome. Some scientists have speculated that Mikulicz Syndrome and Sjögren Syndrome may actually be the same disorder. .
Symptoms of the following disorders can be similar to those of Mikulicz Syndrome. Comparisons may be useful for a differential diagnosis:
Mumps is an acute viral illness that causes painful inflammation and swelling of the salivary glands, including the parotid, submaxillary, sublingual, and buccal glands. At one time, Mumps was a common infectious disease of childhood. However, a vaccine against this disease was developed in 1967. The onset of the illness is marked by headache, loss of appetite, a general feeling of ill health (malaise), and a low to moderate fever. Within 24 hours, the temperature rises to approximately 104.0F and is usually associated with pain and swelling of the glands in front of the ears (parotid) and under the jaw (submaxillary). (For more information on this disorder, choose "Mumps" as your search term in the Rare Disease Database.)
Heerfordt's Syndrome, also known as Uveoparotid fever, is similar to Mikulicz Syndrome and is sometimes the first symptom of another disease, Sarcoidosis. Heerfordt's Syndrome is characterized by swelling of the glands in front of the ears (parotids) and paralysis of one or more of the cranial nerves. The nerves of the face are most commonly affected.
Sjogren Syndrome is an autoimmune disorder characterized by the progressive degeneration of mucous secreting glands, especially those of the mouth (salivary) and eyes (lacrimal). The symptoms of Sjogen Syndrome usually begin suddenly and may include inflammation of the membranes that surround the eyes and the corneas (keratoconjunctivitis). Varying degrees of dryness in the mouth (sicca xerostomia) may cause difficulty swallowing and/or dental disease. (For more information on this disorder, choose "Sjogren" as your search term in the Rare Disease Database.)
Swelling of the glands in front of the ears (parotid swelling) may occur for many different reasons. Painless swelling on both sides of the face may occur without fever and in association with other disorders such as Laennec's Cirrhosis, chronic alcoholism, malnutrition, diabetes mellitus, pregnancy, lactation, and/or Hypertriglyceridemia. Malignant and benign salivary gland tumors can also cause swelling of the salivary glands. Parotid gland enlargement may also be related to the use of certain drugs (e.g., guanethidine or iodine). Obstruction of the duct from the parotid gland to the mouth (Stensen's duct) by a stone (calcification) can also cause swelling of the parotid gland.
Biopsy of one of the swollen glands is key to the diagnosis of Mikulicz syndrome. An ultrasound examination of the area may help to rule out other reasons for gland swelling. Treatment of this disorder is symptomatic. Medical therapies are more productively directed 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.
Some individuals with Mikulicz syndrome may be instructed to follow a soft moist diet. This may help to reduce the pain caused by chewing and swallowing. Other treatment is symptomatic and supportive. .
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
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JOURNAL ARTICLES Goto TK, Shimizu M, Kobayashi I, et al. Lymphoepithelial lesion of the parotid gland. Dentomaxillofac Radiol. 2002;31:198-203.
Sato K, Kawana M, Sato Y, et al. Malignant lymphoma in the head and neck associated with benign lymphoepithelial lesion of the parotid gland. Auris Nasus Larynx. 2002;29:209-14.
Miracco C, Schurfeld K, Cardone C, et al. Benign lymphoepithelial lesion associated with squamous cell carcinoma of the skin: an immunohistochemical and molecular genetic study. J Cutan Pathol. 2002;29:33-37.
Amin MA, Bailey BM, Patel SR. Clinical and radiological evidence to support superficial parotid ectomy as the treatment of choice for chronic parotid sialadenitis: a retrospective study. Br J Oral Maxillofac Surg. 2001;39:348-52.
Carbone A, Gloghini A, Ferlito A. Pathological features of lymphoid proliferations of the salivary glands: lymphoepithelial sialadenitis versus low-grade B-cell lymphoma of the malt type. Ann Otol Rhinol Laryngol. 2000;109(12 Pt1):1170-75.
Tsubota K, Fujita H, Tsuzaka K, et al. Mikulicz's disease and Sjogren's syndrome. Invest Ophthalmol Vis Sci. 2000;41:1666-73.
Ihrler S, Baretton GB, Menauer F, et al. Sjogren's syndrome and MALT lymphomas of the salivary glands: a DNA-cytometric and interphase-cytogenetic study. Mod Pathol. 2000;13:4-12.
Ihrler S, Zietz C, Sendelhofert A, et al. Lymphoepithelial duct lesions in Sjogren-type sialadenitis. Virchows Arch. 1999;434:315-23.
Leung AK, Wong AL, Robson WL, et al. Benign lymphoepithelial lesion (Mikulicz's syndrome) of the submandibular glands in a four-year-old boy. Otolyryngol Head Neck Surg. 1994;111(3 Pt1):302-04.
FROM THE INTERNET Fox RI, Michelson PE, Frosio D. SJÖGREN'S SYNDROME: A GUIDE FOR THE PATIENT. Revised Version; 8.16.02. 76pp. http://dry.org/fox20020816/guide.htm
HONselect. Mikulicz' Disease. Last modified: Jan 29 2003. 2pp. www.hon.ch/cgi-bin/HONselect
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