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Mikulicz Disease

Chronic Dacryoadenitis and Sialadenitis

Mikulicz disease is characterized by the chronic and benign enlargement of the glandular structures situated in the head and neck, with the parotid, lacrimal and other salivary glands amongst the most commonly affected structures.


Presentation

This condition, if primary, has been historically designated as Mikulicz disease. Although benign, this condition is almost always found along with certain other underlying diseases, such as tuberculosis, leukemia, syphilis, hodgkin's disease, sarcoidosis, systemic lupus erythematosus or Sjogren's syndrome. The appropriate terminology for this condition then becomes Mikulicz syndrome [1]. Such designations are now considered outdated and ambiguous. Some authors believe that Mikulicz disease, Mikulicz syndrome as well as Sjogren's syndrome all refer to the same condition- an autoimmune lymphocytic inflammation of the salivary and lacrimal glands due to a hyperactive immune response [2] [3] [4].

Owing to the inflammatory processes occurring in the enlarged parotid and other salivary glands, Mikulicz disease patients are usually affected by xerostomia of a sudden onset that may present as dysphagia or dental problems. The enlargement of the lacrimal glands, on the other hand, may lead to a reduction or absence of tear secretion.

Painless swellings of the parotid and other salivary glands may be found in these patients. Other glands, such as the submaxillary and the buccal glands, may also be involved. Uveitis is another commonly seen clinical sign. This disease is usually long-lasting and recurring fevers may be observed in some patients [5] [6].

Mikulicz disease may also be known by a variety of names including Dacryosialoadenopathia, Mikulicz-Radecki syndrome, Mikulicz-Sjogren's syndrome. Several other conditions, having similar clinical manifestations, should be considered in the differential diagnosis of Mikulicz disease.

Mumps, an acute viral disease of childhood was a leading cause of enlargement of the salivary glands before the advent of prophylactic mass vaccinations. Unlike Mikulicz disease, the inflammation of the glands is usually painful in mumps. The first symptoms may further include malaise, loss of appetite and a low-grade temperature that rises quickly within the first 24 hours.

Heerfordt’s syndrome, characterized by the enlargement of the parotid glands accompanied by the paralysis of the facial nerve should also be excluded. It is an early feature of sarcoidosis.

Salivary gland duct obstruction due to stones may also be a cause for glandular enlargement and should be considered in the differential diagnosis. Other reasons for salivary gland swelling include malnutrition, chronic alcoholism, pregnancy and the use of certain medications such as iodine. Salivary gland tumors, both benign and malignant, should also be ruled out while making a diagnosis [7] [8].

Mediastinal Lymphadenopathy
  • A chest computerized tomography showed mediastinal lymphadenopathy, with low metabolic activity on the position emission tomography.[clinexprheumatol.org]
Dental Caries
  • Xerostomia Dental caries Early loss of teeth Dry & smooth oral mucosa Cracking & Fissuring at corner of mouth.Management Good oral hygiene. 10. Hyperplasia Cause- Hormonal MetabolicClinical features Site minor salivary glands of palate.[slideshare.net]
  • In addition to chronic dryness, the patients have troublesome intraoral soft tissue problems that include rampant dental caries and difficulty with dentures due to dryness.[dry.org]
Lacrimal Gland Enlargement
  • Historically, bilateral parotid and lacrimal gland enlargement was characterized by the term Mikulicz's disease if the enlargement appeared apart from other diseases.[en.wikipedia.org]
  • Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs. The exact cause of Mikulicz syndrome is not known. Some scientists believe that Mikulicz syndrome should be considered a form of Sjögren syndrome.[rarediseases.org]
Absent Lacrimation
  • - A dictionary of medical eponyms Related people Henrik Samuel Conrad Sjögren Jan Mikulicz-Radecki Benign and chronic dacryoadenitis with bilateral painless swelling of lacrimal and salivary glands and decreased or absent lacrimation.[whonamedit.com]
Hyperactivity
  • Some authors believe that Mikulicz disease, Mikulicz syndrome as well as Sjogren's syndrome all refer to the same condition- an autoimmune lymphocytic inflammation of the salivary and lacrimal glands due to a hyperactive immune response.[symptoma.com]
Abnormal Involuntary Movement
  • involuntary movement scale ) 免疫グロブリンG ( immunoglobulin G ) WebLSDに未収録の専門用語(用法)は "新規対訳" から投稿できます。[life-science-dictionary.com]

Workup

Mikulicz disease is suspected in patients presenting with a simultaneous enlargement of the parotid and lacrimal glands.

Biopsy may be required to establish the reason for the enlargement of these glands. Histological findings in Mikulicz disease include lymphocytic infiltration in the salivary and lacrimal glands. Autoantibodies, such as anti-Ro, anti-La, antinuclear antibodies (ANA) and rheumatoid factor may also be detected in the serum.

Dry eye may be objectively diagnosed by certain tear flow dynamic tests, including the schirmer test, cotton thread test, clearance test or the tear break-up time test. Changes in the ocular surface may be detected via staining with the rose bengal or fluorescein stains [9] [10].

Treatment

  • A brief description of the disease and its treatment is also presented.[ncbi.nlm.nih.gov]
  • MD sometimes mimics intraorbital tumors such as malignant lymphoma but responds well to corticosteroid treatment. Thus it is important to recognize the clinical and radiographic features of MD. Copyright 2017 Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • (a) Serum concentrations of both IgG1 and IgG4 from patients with MD decreased after treatment.[doi.org]
  • As for the treatment of IgG4-RD, oral prednisolone is considered the primary treatment in the presence of symptomatic disease with organ involvement [ 13 ].[synapse.koreamed.org]
  • The concentration ( /-s.d.) of IgG4 in MD decreased to 254.0 /- 50.3 mg/dl (P 0.05) after glucocorticoid treatment.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis is usually good; rarely this condition may devolve into lymphoma, or could actually represent 'occult' lymphoma from the outset.[en.wikipedia.org]
  • Controversial points regarding Mikulicz's disease Prognosis and relationship with lymphoproliferative diseases The long-term prognosis of MD is unknown.[link.springer.com]
  • […] with Masson trichrome (bottom left) Images on the right show slices with immunohistochemical markers 100 revealing numerous mature lymphocytes, CD20, CD4 and IgG4 producing plasma cells. a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):332–335 335 Prognosis[kundoc.com]
  • Prognosis of IgG4-related disease is variable. It may spontaneously resolve or persist, with remitting and relapsing symptoms.[dermnetnz.org]
  • Occurrence and prognosis of extranodal lymphomas. Cancer, 1 972;29 :252-260. 14. Nime, F.A., Cooper, H.S. and Eggleston, J.C. Primary malignant lymphomas of the salivary glands. Cancer, 1 976;37:906-912. 15. Shikhani, A., Samara, M, Allam, C.[jpma.org.pk]

Etiology

  • Schaffer and Jacobsen in 1927 reviewed the subject comprehensively and offered an etiologic classification. Sjögren's syndrome is entered as a separate entity under Henrik Samuel Conrad Sjögren, Swedish ophthalmologist, 1899-1986.[whonamedit.com]
  • Kumar KEYWORDS: Kimura’s Disease , Eosinophilia , Neck Swelling JOURNAL NAME: International Journal of Otolaryngology and Head & Neck Surgery , Vol.3 No.4 , July 11, 2014 ABSTRACT: Kimura’s disease (KD) is a chronic inflammatory disorder with unknown etiology[scirp.org]
  • It has also been suggested that dacryoadenitis from EBV responds well to systemic steroids. 21 Inflammatory etiologies should be managed based on the specific treatment for the etiology.[reviewofophthalmology.com]
  • Although the etiology and mechanism of AIP are still unknown, overproduction of Th2 and regulatory cytokines may play an important role [ 11 , 12 ].[basicmedicalkey.com]
  • Etiology Drugs like sialogogues Local factors ANUG,erythema multiforme Systemic like paralysis Misc. like metal poisoning 14.[slideshare.net]

Epidemiology

  • Immunosurveillance and cancer: epidemiological evidence. Br Med J. 1970 Nov 14; 4 (5732):420–422. [ PMC free article ] [ PubMed ] [ Google Scholar ] GODWIN JT.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature.[ncbi.nlm.nih.gov]
  • Conflicting reports have implicated Th1 cells and Th2 cells in disease pathophysiology [8]. Within the organs, increased CD4-positive T cells lead to activation of innate immune cells, which secrete cytokines and interleukins.[journals.viamedica.pl]
  • Kazushige Uchida and Kazuichi Okazaki , Clinical and pathophysiological aspects of type 1 autoimmune pancreatitis , Journal of Gastroenterology , 10.1007/s00535-018-1440-8 , 53 , 4 , (475-483) , (2018) .[doi.org]

Prevention

  • Thus, IgG4 is supposed to prevent large immune complex formation and shows inhibitory effect against allergic reactions.[e-ijd.org]
  • Also, therapies are directed at improving symptoms, preventing the complications (such as dental caries, oral candida, or corneal damage) and preventing disease progression. A.[dry.org]
  • Sánchez Fontán, Chairman of the Board of the Association to Prevent Blindness in Mexico I. A.P.-Hospital Dr. Luis Sánchez Bulnes. Thanks for all the opportunities offered to us year after year. Fig. 6 – Clinical picture of patient outcome.[kundoc.com]
  • Xerostomia Dryness of mouth.Etiology Radiation induced Drug induced NutritionalClinical features Increase thirst Dry leathery tongue Difficulty in speech, swallowing & eating dry food Burning sensation Blurred vision Fissuring of tongue.Management Preventive[slideshare.net]

References

Article

  1. Ihrler S, Harrison J. 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. 2005;100(3):334-9.
  2. Yousem D, Kraut M, Chalian A. Major salivary gland imaging. Radiology. 2000;216(1):19-29.
  3. Morgan W, Castleman B. A clinicopathologic study of “Mikulicz’s Disease. Am J Pathol. 1953;29:471-503.
  4. Morgan W. The probable systemic nature of Mikulicz’s disease and its relation to Sjögren’s syndrome. N Engl J Med. 1954;251:5-10.
  5. Lemp M. Report of the National Eye Institute/industry workshop on clinical trials in dry eyes. CLAO J. 1995;21:221-232.
  6. Fox R, Robinson C, Curd J, Kozin F, Howell F. Sjögren’s syndrome: proposed criteria for classification. Arthritis Rheum. 1986;29:577-583.
  7. Mikulicz J. Ueber eine eigenartige symmetrische Erkrankung der Tranen und Mundspeicheldrusen (Concerning a peculiar symmetrical disease of the lacrimal and salivary glands). Medical Classics. 1937;2:165-186.
  8. Miracco C, Schurfeld K, Cardone C, et al. Benign lymphoepithelial lesion associated with squamous cell carcinoma of the skin: an immunohistochemical and molecular genetic study. J Cutan Pathol. 2002;29:33-37.
  9. Toda I, Shimazaki J, Tsubota K. Dry eye with only decreased break-up time is sometimes associated with allergic conjunctivitis. Ophthalmology. 1995;102:302-309.
  10. Tsubota K, Fujita H, Tsuzaka K, al. e. Mikulicz’s disease and Sjögren’s syndrome. Invest Ophthalmol Vis ScI. 2000;41:1666-73.

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Last updated: 2018-06-22 07:50