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 Such designations are now considered outdated and ambiguous.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].

• 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]

• 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]

  The fever may be accompanied by dry eyes, diminished tear production (lacrimation), and inflammation of various parts of the eyes (uveitis). Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs. [rarediseases.org]

  The associations of orbital involvement (lacrimal gland enlargement and/or intraorbital masses) and cranial nerve enlargement, and of enlargement of both cranial nerve and pituitary gland, were most common (4 cases each). [ajnr.org]

  Previously, bilateral lacrimal gland enlargement associated with salivary gland enlargement was referred to as Mikulicz’s disease, before the discovery of IgG4-RD as a unique clinical entity. [25] Compared to patients with traditional idiopathic orbital [eyewiki.aao.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]

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].

• Antinuclear Autoantibodies

  The following factors having a possible association with the presence or absence of MD were investigated: sex; serum IgG and IgG4 levels; the presence or absence of antinuclear autoantibodies, jaundice, diabetes mellitus, swollen duodenal papilla, diffuse [ncbi.nlm.nih.gov]

• Thrombocytosis

  In hyper-IL6 syndromes, the levels of IgG, IgA, IgM, and CRP are mostly elevated, and thrombocytosis, anemia, hypoalbuminemia and hypocholestrolemia are observed due to the effects of the high IL-6 levels. [synapse.koreamed.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]

(a) Serum concentrations of both IgG1 and IgG4 from patients with MD decreased after treatment. [doi.org]

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. [ncbi.nlm.nih.gov]

Treatment of this disorder is symptomatic. Medical therapies are more productively directed toward the treatment of any underlying disease. [rarediseases.org]

Prognosis is usually good; rarely this condition may devolve into lymphoma, or could actually represent 'occult' lymphoma from the outset. [en.wikipedia.org]

What is the prognosis of IgG4-related disease? Prognosis of IgG4-related disease is variable. It may spontaneously resolve or persist, with remitting and relapsing symptoms. [dermnetnz.org]

Smoking habits and a history of systemic disease also have been associated with poor prognosis of the disease. [19] To date, malignant transformation of Kimura disease has not been described in the literature. Kimm HT, Szeto C. [emedicine.medscape.com]

Controversial points regarding Mikulicz's disease Prognosis and relationship with lymphoproliferative diseases The long-term prognosis of MD is unknown. [link.springer.com]

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]

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]

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]

Sudesh 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]

The theories of etiology are diverse. Many authors are convinced that sialoliths or scarring of the ducts cause stasis of salivary flow and predispose the gland to infection is the etiology, but this is probably true for only a minority of cases. [emedicine.medscape.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]

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]

Epidemiology Frequency United States Kimura disease has rarely been reported in the United States. International The exact prevalence of Kimura disease is not known. [emedicine.medscape.com]

Epidemiology in the pediatric population is also unknown, as there are few reported cases among children. [eyewiki.aao.org]

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]

Kimura disease may represent a primary inflammatory process with secondary vascular proliferation. [5, 6] Reports have described both diseases presenting simultaneously. [7] Pathophysiology The pathophysiology of Kimura disease remains unknown. [emedicine.medscape.com]

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]

Pathophysiology The etiology and pathogenesis of IgG4-ROD is currently unknown. Generally, pathogenesis is thought to revolve around humoral and cell-mediated immunity. [eyewiki.aao.org]

Thus, IgG4 is supposed to prevent large immune complex formation and shows inhibitory effect against allergic reactions. [e-ijd.org]

The spectrum varies from mild and infrequent attacks to episodes so frequent that they prevent regular school attendance. [emedicine.medscape.com]

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]

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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.