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a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/02 04:31:34」(JST)

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Mikulicz syndrome redirects here.

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.

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Today, the terms "Mikulicz's disease" and "Mikulicz's syndrome" are viewed as ambiguous and outdated by some sources.^[3]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

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.

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.

References

^ 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.

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

UpToDate Contents

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English Journal

Unusual Multiorgan Immunoglobulin G4 (IgG4) Inflammation: Autoimmune Pancreatitis, Mikulicz Syndrome, and IgG4 Mastitis.

Dítě P, Trna J, Kinkor Z, Novotný I, Lata J, Kianička B, Hermanová M.SourceDepartment of Internal Medicine and Hepatogastroenterology, University Hospital Brno, Masaryk University Faculty of Medicine, Brno, Czech Republic. ; Department of Internal Medicine, University of Ostrava Faculty of Medicine, Ostrava, Czech Republic.
Gut and liver.Gut Liver.2013 Sep;7(5):621-4. doi: 10.5009/gnl.2013.7.5.621. Epub 2013 Sep 11.
Autoimmune pancreatitis (AIP) type 1 is commonly associated with simultaneous involvement of extrapancreatic organs. Sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, Sjögren syndrome, and other extrapancreatic lesions are often observed concurrently with AIP. High levels of immunoglo
PMID 24073323

IgG4-related disease.

Palazzo E, Palazzo C, Palazzo M.SourceService de rhumatologie, hôpital Bichat, Paris, France. Electronic address: elisabeth.palazzo@bch.aphp.fr.
Joint, bone, spine : revue du rhumatisme.Joint Bone Spine.2013 Jul 9. pii: S1297-319X(13)00153-X. doi: 10.1016/j.jbspin.2013.06.001. [Epub ahead of print]
The term "IgG4-related disease" encompasses several disorders described many years ago under various designations depending on the organ or system involved (e.g., Mikulicz syndrome, Riedel's thyroiditis, and retroperitoneal fibrosis). The clinical presentation varies widely, as one or more organs ma
PMID 23849464