Benign lymphoepithelial lesion or Mikulicz' disease is a type of

benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...

enlargement of the

parotid The parotid gland is a major salivary gland in many animals. In humans, the two parotid glands are present on either side of the mouth and in front of both ears. They are the largest of the salivary glands. Each parotid is wrapped around the ma ...

and/or

lacrimal gland The lacrimal glands are paired exocrine glands, one for each eye, found in most terrestrial vertebrates and some marine mammals, that secrete the aqueous layer of the tear film. In humans, they are situated in the upper lateral region of each or ...

s. This

pathologic ''Pathologic'' ( rus, Мор. Утопия, Mor. Utopiya, ˈmor ʊˈtopʲɪjə, , More. Utopia – a pun on Thomas More's ''Utopia'' and the Russian word for "plague") is a 2005 role-playing and survival game developed by Russian studio Ice-Pic ...

state is sometimes, but not always, associated with Sjögren's syndrome.

Presentation

Benign lymphoepithelial lesion is most likely to occur in

adult An adult is a human or other animal that has reached full growth. In human context, the term ''adult'' has meanings associated with social and legal concepts. In contrast to a " minor", a legal adult is a person who has attained the age of major ...

s around 50 years of age. Approximately 60–80% of those affected are

female Female (Venus symbol, symbol: ♀) is the sex of an organism that produces the large non-motile ovum, ova (egg cells), the type of gamete (sex cell) that fuses with the Sperm, male gamete during sexual reproduction. A female has larger gamet ...

. The gland affected has a diffuse swelling. The swelling can be

asymptomatic In medicine, any disease is classified asymptomatic if a patient tests as carrier for a disease or infection but experiences no symptoms. Whenever a medical condition fails to show noticeable symptoms after a diagnosis it might be considered asy ...

, but mild

pain Pain is a distressing feeling often caused by intense or damaging stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sensory and emotional experience associated with, or resembling that associated with, ...

can also be associated. This condition occurs often in those with

HIV The human immunodeficiency viruses (HIV) are two species of ''Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the immune ...

infection. Most cases of benign lymphoepithelial lesions appear in conjunction with Sjögren's syndrome. When Sjögren's syndrome is present, the swelling is usually

bilateral Bilateral may refer to any concept including two sides, in particular: * Bilateria, bilateral animals *Bilateralism, the political and cultural relations between two states *Bilateral, occurring on both sides of an organism ( Anatomical terms of ...

. Otherwise, the affected glands are usually only on one side of the body. In many cases, a

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a diseas ...

is needed to distinguish benign lymphoepithelial lesions from sialadenosis (sialosis).

Locations

In 80% of cases, the parotid gland is affected. Lacrimal glands are also affected.

Histology

There is a marked lymphoplasmacytic infiltration. Lymphoid follicles surround solid epithelial nests, giving rise to the 'epimyoepithelial islands', that are mainly composed of ductal cells with occasional myoepithelial cells. Excess hyaline basement membrane material is deposited between cells, and there is also acinar atrophy and destruction.

Treatment

Treatment usually consists of observation unless the patient has concern, there is pain, drainage, or other symptoms related to the lesion. Surgical removal of the affected gland would be recommended in those cases. Another treatment option would be aspiration, which can be repeated multiple times. This is commonly performed in those who are debilitated or in those whose benefit from surgery would be outweighed by the risks. Prognosis is usually good; rarely this condition may devolve into lymphoma, or could actually represent 'occult' lymphoma from the outset.

Eponym

Historically, bilateral parotid and lacrimal

gland In animals, a gland is a group of cells in an animal's body that synthesizes substances (such as hormones) for release into the bloodstream (endocrine gland) or into cavities inside the body or its outer surface (exocrine gland). Structure De ...

enlargement was characterized by the term Mikulicz's disease if the enlargement appeared apart from other diseases. If it was secondary to another disease, such as

tuberculosis Tuberculosis (TB) is an infectious disease usually caused by '' Mycobacterium tuberculosis'' (MTB) bacteria. Tuberculosis generally affects the lungs, but it can also affect other parts of the body. Most infections show no symptoms, in ...

sarcoidosis Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly af ...

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlar ...

, and Sjögren's syndrome, the term used was Mikulicz's syndrome. Both names derive from

Jan Mikulicz-Radecki Jan Mikulicz-Radecki (german: Johann Freiherr von Mikulicz-Radecki) was a German-Polish-Austrian surgeon who worked mainly in the German Empire. He was born on 16 May 1850 in Czerniowce in the Austrian Empire (present-day Chernivtsi in Ukraine) ...

, the

Polish Polish may refer to: * Anything from or related to Poland, a country in Europe * Polish language * Poles, people from Poland or of Polish descent * Polish chicken *Polish brothers (Mark Polish and Michael Polish, born 1970), American twin screenwr ...

surgeon In modern medicine, a surgeon is a medical professional who performs surgery. Although there are different traditions in different times and places, a modern surgeon usually is also a licensed physician or received the same medical training as ...

best known for describing these conditions. In more recent times, the terms "Mikulicz's disease" and "Mikulicz's syndrome" were viewed as ambiguous and outdated by some sources. Today Mikulicz's disease is considered to be a subtype of

IgG4-related disease IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and ...

, usually accompanied by involvement of one or more other organs in the body.

References

;Bibliography * Kahn, Michael A. Basic Oral and Maxillofacial Pathology. Volume 1. 2001. * Regezi, Joseph A. Oral Pathology: Clinical Pathologic Correlations. 4th ed. 2002.

External links

Mikulicz syndrome
on The National Organization for Rare Disorders (NORD)
Benign lymphoepithelial lesions
on Radiopedia {{Oral pathology Salivary gland pathology IgG4-related disease