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$Unique_ID{BRK04003}
$Pretitle{}
$Title{Mikulicz Syndrome}
$Subject{Mikulicz Syndrome Dacryosialoadenopathy Dacryosialoadenopathia
Mikulicz-Sjogren Syndrome Mikulicz-Radecki Syndrome von Mikulicz Syndrome
Mikulicz Syndrome}
$Volume{}
$Log{}
Copyright (C) 1986 National Organization for Rare Disorders, Inc.
205:
Mikulicz Syndrome
** IMPORTANT **
It is possible the main title of the article (Mikulicz Syndrome) is not
the name you expected. Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Dacryosialoadenopathy
Dacryosialoadenopathia
Mikulicz-Sjogren Syndrome
Mikulicz-Radecki Syndrome
von Mikulicz Syndrome, also known as Mikulicz Syndrome
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Mikulicz Syndrome is a benign chronic lymphocytic infiltration and
enlargement of the tonsils, and glands near the ear (parotid), beneath the
upper jaw bone (submaxillary), tear (lacrimal), and salivary glands. This
condition causes excessive dryness of the mouth and eyes.
Symptoms
Mikulicz Syndrome is characterized by dryness of the mouth which may cause
difficulty in swallowing and tooth decay, absent or decreased tears, and
blurred vision. Symmetric, painless, hard swellings (tumefactions) of the
tear and salivary glands occur and recur during the course of the disease,
which waxes and wanes.
Causes
The causes of Mikulicz Syndrome are unknown, although it is suspected to be
an autoimmune disorder. The disease may be associated with chronic
lymphocytic leukemia, lymphosarcoma, Hodgkin's Disease, tuberculosis,
sarcoidosis or systemic lupus erythematosus. It can also be part of a
familial disorder which often occurs in combination with Sjogren Syndrome.
Some scientists have speculated that Mikulicz Syndrome and Sjogren Syndrome
may actually be the same disorder.
Related Disorders
Sjogren Syndrome is a degeneration of the tear and salivary glands which may
be associated with arthritis. Malignant and benign salivary gland tumors can
also cause swelling of the salivary glands. Mumps is an acute, contagious,
generalized viral infection usually causing the painful enlargement of the
salivary glands, most commonly those near the ear (the parotids). Parotid
enlargement can also be drug-related (e.g., caused by iodides or
guanethidine). Obstruction of the duct from the parotid gland to the mouth
(Stensen's duct) by a stone can also cause swelling of the parotid gland.
Therapies: Standard
Diagnosis of Mikulicz Syndrome is accomplished by means of a biopsy.
Treatment of this disorder is symptomatic. A soft moist diet can reduce the
pain caused by chewing and swallowing. In severe cases, artificial saliva
can be used to moisten the mouth, and, in most cases, artificial tears should
be used to keep the eyes moist and thus avoid infection.
Therapies aimed at treating the underlying cause of the disease may also
be initiated. Examples include radiation therapy if the primary condition is
Hodgkin's Disease or a lymphosarcoma, antibiotics if the underlying disease
is syphilis or tuberculosis, and corticosteroids are applied if the cause is
sarcoidosis. If lymphocytic leukemia is the underlying cause, it is usually
treated with cancer chemotherapy drugs. If the primary condition is Systemic
Lupus Erythematosus (SLE) or Sjogren Syndrome, anti-inflammatory and anti-
malarial drugs are prescribed for treating the mild forms of these disorders
and corticosteroids are used when the disease is more severe. (For more
information of Systemic Lupus Erythematosus and Sjogren Syndrome, please
choose "Lupus" and "Sjogren" as your search terms in the Rare Disease
database.)
Therapies: Investigational
This disease entry is based upon medical information available through May
1990. Since NORD's resources are limited, it is not possible to keep every
entry in the Rare Disease Database completely current and accurate. Please
check with the agencies listed in the Resources section for the most current
information about this disorder.
Resources
For more information on Mikulicz Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Institute of Dental Research
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4261
Sjogren Syndrome Foundation
382 Main St.
Port Washington, NY 11050
(516) 767-2866
National Sjogren Syndrome Association
3201 W. Evans Dr.
Phoenix, AZ 85023
(800) 385-6772 or (602) 993-7227
References
THE MERCK MANUAL, 15th ed., Robert Berkow, M.D., ed in chief, published by
Merck, Sharp & Dohme Research Labs, Rahway, NJ, 1987. Pp. 2040, 2323.