Benign lymphoepithelial lesion
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- Mikulicz syndrome redirects here.
| Benign lymphoepithelial lesion | |
|---|---|
| Classification and external resources | |
| Specialty | Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value). |
| ICD-10 | K11.8 |
| ICD-9-CM | 527.1 |
| DiseasesDB | 8218 |
| Patient UK | Benign lymphoepithelial lesion |
| MeSH | D008882 |
Benign lymphoepithelial lesion is a type of benign enlargement of the parotid and/or lacrimal glands. This pathologic state is sometimes, but not always, associated with Sjögren's syndrome.
Contents
Eponym
Historically, bilateral parotid and lacrimal gland 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, sarcoidosis, lymphoma, and Sjögren's syndrome, the term used was Mikulicz's syndrome. Both names derive from Jan Mikulicz-Radecki, the Polish surgeon best known for describing these conditions.[1][2]
Today, the terms "Mikulicz's disease" and "Mikulicz's syndrome" are viewed as ambiguous and outdated by some sources.[3]
Locations
In 80% of cases, the parotid gland is affected. Lacrimal glands are also affected.
Characteristic
Benign lymphoepithelial lesion is most likely to occur in adults around 50 years of age. There is a predilection for gender with 60–80% being female. The gland affected has a diffuse swelling. The swelling can be asymptomatic, but mild pain can also be associated. There is a preponderance of this disease in those who suffer from HIV 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. Otherwise, the affected glands are usually only on one side of the body.
In many cases, a biopsy is needed to distinguish benign lymphoepithelial lesions from sialadenosis (sialosis).
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.
See also
References
- ↑ synd/2087 at Who Named It? - "Mikulicz's disease"
- ↑ synd/2088 at Who Named It? - "Mikulicz's syndrome"
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- Bibliography
- Kahn, Michael A. Basic Oral and Maxillofacial Pathology. Volume 1. 2001.
- Regezi, Joseph A. Oral Pathology: Clinical Pathologic Correlations. 4th ed. 2002.
Further reading
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External links
- Mikulicz syndrome on The National Organization for Rare Disorders (NORD)
- Benign lymphoepithelial lesions on Radiopedia
