Benign lymphoepithelial lesion is most likely to occur in adults around 50 years of age. Approximately 60–80% of those affected are female. The gland affected has a diffuse swelling. The swelling can be asymptomatic, but mild pain can also be associated. This condition occurs often in those with HIV infection.[citation needed]
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.[citation needed]
In many cases, a biopsy is needed to distinguish benign lymphoepithelial lesions from sialadenosis (sialosis).
Locationsedit
In 80% of cases, the parotid gland is affected. Lacrimal glands are also affected.[citation needed]
Histologyedit
Section from salivary gland showing dense lymphoid infiltrate around and within ductal epithelium-Lympho epithelial lesion (H&E,100X)
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.[citation needed]
Treatmentedit
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.[citation needed]
Eponymedit
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 Polishsurgeon best known for describing these conditions.[1][2]
In more recent times, the terms "Mikulicz's disease" and "Mikulicz's syndrome" were viewed as ambiguous and outdated by some sources.[3]
Today Mikulicz's disease is considered to be a subtype of IgG4-related disease, usually accompanied by involvement of one or more other organs in the body.[4]
^John H. Stone; Arezou Khosroshahi; Vikram Deshpande; John K. C. Chan; J. Godfrey Heathcote; Rob Aalberse; Atsushi Azumi; Donald B. Bloch; William R. Brugge; Mollie N. Carruthers; Wah Cheuk; Lynn Cornell; Carlos Fernandez-Del Castillo; Judith A. Ferry; David Forcione; Günter Klöppe; Daniel L. Hamilos; Terumi Kamisawa; Satomi Kasashima; Shigeyuki Kawa; Mitsuhiro Kawano; Yasufumi Masaki; Kenji Notohara; Kazuichi Okazaki; Ji Kon Ryu; Takako Saeki; Dushyant Sahani; Yasuharu Sato; Thomas Smyrk; James R. Stone; Masayuki Takahira; Hisanori Umehara; George Webster; Motohisa Yamamoto; Eunhee Yi; Tadashi Yoshino; Giuseppe Zamboni; Yoh Zen; Suresh Chari (October 2012). "Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations". Arthritis & Rheumatism. 64 (10): 3061–3067. doi:10.1002/art.34593. PMC5963880. PMID 22736240.
Bibliography
Kahn, Michael A. Basic Oral and Maxillofacial Pathology. Volume 1. 2001.
Regezi, Joseph A. Oral Pathology: Clinical Pathologic Correlations. 4th ed. 2002.
Further readingedit
Kahn, LB (Jan 1979). "Benign lymphoepithelial lesion (Mikulicz's disease) of the salivary gland: an ultrastructural study". Human Pathology. 10 (1): 99–104. doi:10.1016/S0046-8177(79)80077-5. PMID 428999.
Lee S, Tsirbas A, McCann J, Goldberg R (2006). "Mikulicz's disease: a new perspective and literature review". Eur J Ophthalmol. 16 (2): 199–203. doi:10.1177/112067210601600201. PMID 16703534. S2CID 24906450.
Azzopardi, JG; Evans, DJ (Nov 1971). "Malignant lymphoma of parotid associated with Mikulicz disease (benign lymphoepithelial lesion)". J Clin Pathol. 24 (8): 744–752. doi:10.1136/jcp.24.8.744. PMC477147. PMID 4943296.
Yamamoto, M; Harada, S; Ohara, M; Suzuki, C; Naishiro, Y; Yamamoto, H; Takahashi, H; Imai, K (Feb 2005). "Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome". Rheumatology. 44 (2): 227–34. doi:10.1093/rheumatology/keh447. PMID 15509627.
Delaney, William E.; Balogh, Károly (1966). "Carcinoma of the parotid gland associated with benign lymphoepithelial lesion (Mikulicz's disease) in Sjögren's syndrome". Cancer. 19 (6): 853–860. doi:10.1002/1097-0142(196606)19:6<853::AID-CNCR2820190617>3.0.CO;2-N. PMID 5939056.
External linksedit
Mikulicz syndrome on The National Organization for Rare Disorders (NORD)
Benign lymphoepithelial lesions on Radiopedia
November 27, 2023
benign, lymphoepithelial, lesion, mikulicz, disease, type, benign, enlargement, parotid, lacrimal, glands, this, pathologic, state, sometimes, always, associated, with, sjögren, syndrome, other, namesmikulicz, diseasespecialtygastroenterology, contents, presen. 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 Sjogren s syndrome Benign lymphoepithelial lesionOther namesMikulicz diseaseSpecialtyGastroenterology Contents 1 Presentation 2 Locations 3 Histology 4 Treatment 5 Eponym 6 See also 7 References 8 Further reading 9 External linksPresentation editBenign lymphoepithelial lesion is most likely to occur in adults around 50 years of age Approximately 60 80 of those affected are female The gland affected has a diffuse swelling The swelling can be asymptomatic but mild pain can also be associated This condition occurs often in those with HIV infection citation needed Most cases of benign lymphoepithelial lesions appear in conjunction with Sjogren s syndrome When Sjogren s syndrome is present the swelling is usually bilateral Otherwise the affected glands are usually only on one side of the body citation needed In many cases a biopsy is needed to distinguish benign lymphoepithelial lesions from sialadenosis sialosis Locations editIn 80 of cases the parotid gland is affected Lacrimal glands are also affected citation needed Histology edit nbsp Section from salivary gland showing dense lymphoid infiltrate around and within ductal epithelium Lympho epithelial lesion H amp E 100X 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 citation needed Treatment editTreatment 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 citation needed Eponym editHistorically 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 Sjogren 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 In more recent times the terms Mikulicz s disease and Mikulicz s syndrome were viewed as ambiguous and outdated by some sources 3 Today Mikulicz s disease is considered to be a subtype of IgG4 related disease usually accompanied by involvement of one or more other organs in the body 4 See also editLymphoepithelial lesionReferences edit synd 2087 at Who Named It Mikulicz s disease synd 2088 at Who Named It Mikulicz s syndrome Ihrler S Harrison J 2005 Mikulicz s disease and Mikulicz s syndrome analysis of the original case report of 1892 in the light of current knowledge identifies a MALT lymphoma Oral Surg Oral Med Oral Pathol Oral Radiol Endod 100 3 334 9 doi 10 1016 j tripleo 2005 01 016 PMID 16122662 John H Stone Arezou Khosroshahi Vikram Deshpande John K C Chan J Godfrey Heathcote Rob Aalberse Atsushi Azumi Donald B Bloch William R Brugge Mollie N Carruthers Wah Cheuk Lynn Cornell Carlos Fernandez Del Castillo Judith A Ferry David Forcione Gunter Kloppe Daniel L Hamilos Terumi Kamisawa Satomi Kasashima Shigeyuki Kawa Mitsuhiro Kawano Yasufumi Masaki Kenji Notohara Kazuichi Okazaki Ji Kon Ryu Takako Saeki Dushyant Sahani Yasuharu Sato Thomas Smyrk James R Stone Masayuki Takahira Hisanori Umehara George Webster Motohisa Yamamoto Eunhee Yi Tadashi Yoshino Giuseppe Zamboni Yoh Zen Suresh Chari October 2012 Recommendations for the nomenclature of IgG4 related disease and its individual organ system manifestations Arthritis amp Rheumatism 64 10 3061 3067 doi 10 1002 art 34593 PMC 5963880 PMID 22736240 BibliographyKahn Michael A Basic Oral and Maxillofacial Pathology Volume 1 2001 Regezi Joseph A Oral Pathology Clinical Pathologic Correlations 4th ed 2002 Further reading editKahn LB Jan 1979 Benign lymphoepithelial lesion Mikulicz s disease of the salivary gland an ultrastructural study Human Pathology 10 1 99 104 doi 10 1016 S0046 8177 79 80077 5 PMID 428999 Lee S Tsirbas A McCann J Goldberg R 2006 Mikulicz s disease a new perspective and literature review Eur J Ophthalmol 16 2 199 203 doi 10 1177 112067210601600201 PMID 16703534 S2CID 24906450 Tsubota K Fujita H Tsuzaka K Takeuchi T Jun 2000 Mikulicz s disease and Sjogren s syndrome Investigative Ophthalmology amp Visual Science 41 7 1666 73 PMID 10845583 Azzopardi JG Evans DJ Nov 1971 Malignant lymphoma of parotid associated with Mikulicz disease benign lymphoepithelial lesion J Clin Pathol 24 8 744 752 doi 10 1136 jcp 24 8 744 PMC 477147 PMID 4943296 Yamamoto M Harada S Ohara M Suzuki C Naishiro Y Yamamoto H Takahashi H Imai K Feb 2005 Clinical and pathological differences between Mikulicz s disease and Sjogren s syndrome Rheumatology 44 2 227 34 doi 10 1093 rheumatology keh447 PMID 15509627 Delaney William E Balogh Karoly 1966 Carcinoma of the parotid gland associated with benign lymphoepithelial lesion Mikulicz s disease in Sjogren s syndrome Cancer 19 6 853 860 doi 10 1002 1097 0142 196606 19 6 lt 853 AID CNCR2820190617 gt 3 0 CO 2 N PMID 5939056 External links editMikulicz syndrome on The National Organization for Rare Disorders NORD Benign lymphoepithelial lesions on Radiopedia Retrieved from https en wikipedia org w index php title Benign lymphoepithelial lesion amp oldid 1170971163, wikipedia, wiki, book, books, library,
