Presentation and Demographics
- Benign lymphoepithelial lesion occurs most commonly in adults around 50 years of age.
- Approximately 60-80% of those affected are female.
- The gland affected shows diffuse swelling.
- The swelling can be asymptomatic or associated with mild pain.
- This condition is often seen in individuals with HIV infection.
Locations
- In 80% of cases, the parotid gland is affected.
- Lacrimal glands can also be affected.
Histology
- Histological examination shows a marked lymphoplasmacytic infiltration.
- Lymphoid follicles surround solid epithelial nests, forming epimyoepithelial islands.
- Excess hyaline basement membrane material is deposited between cells.
- Acinar atrophy and destruction can be observed.
Treatment
- Observation is usually the preferred approach unless the patient has concerns or experiences pain, drainage, or other symptoms related to the lesion.
- Surgical removal of the affected gland may be recommended in certain cases.
- Aspiration can be performed as an alternative treatment option, which can be repeated multiple times.
- Aspiration is commonly performed in debilitated individuals or when the risks of surgery outweigh the benefits.
- Prognosis is generally good, but in rare cases, the condition may progress to lymphoma or represent occult lymphoma from the beginning.
Eponym
- Historically, bilateral parotid and lacrimal gland enlargement was referred to as Mikuliczs disease when it appeared independently from other diseases.
- If the enlargement was secondary to another disease, such as tuberculosis, sarcoidosis, lymphoma, or Sjögrens syndrome, it was called Mikuliczs syndrome.
- Both terms are derived from Jan Mikulicz-Radecki, a Polish surgeon who described these conditions.
- However, Mikuliczs disease and Mikuliczs syndrome are considered ambiguous and outdated by some sources.
- Today, Mikuliczs disease is classified as a subtype of IgG4-related disease, often involving multiple organs in the body.
Benign lymphoepithelial lesion or Mikulicz' disease 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.
| Benign lymphoepithelial lesion | |
|---|---|
| Other names | Mikulicz' disease |
 | |
| Specialty | Gastroenterology  |
