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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 Such designations are now considered outdated and ambiguous.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].

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
  • Eponym [ edit ] 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 was observed in 8 cases. Other lesions included orbital pseudotumor in 5, pituitary enlargement in 5, and cranial nerve enlargement in 7; the infraorbital nerve was involved in 4.[ajnr.org]
  • Role of Th2 cells in IgG4-related lacrimal gland enlargement. Int Arch Allergy Immunol 2010;152(Suppl. 1):47-53. Yamada K, Kawano M, Inoue R et al.[eyewiki.aao.org]

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.[ncbi.nlm.nih.gov]
  • 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]
  • Surgery and medical therapy are useful for treatment. We present a 14 years old male developed a unilateral cervical mass associated with KD. It was a recurrence of disease.[scirp.org]
  • Profil hormonalny Investigation Before Treatment After Treatment at 3 Months Free T4 (0.8-1.8 ng/dL) 0.39 0.928 Thyroid Stimulating Hormone (0.35-5.5 uIU/mL) 0.083 3.28 Prolactin (5-25 ng/mL) 49.04 14.97 Cortisol (5-25 ugm/dL) 0.77 1.2 ACTH ( 5-20 pg[journals.viamedica.pl]

Prognosis

  • Controversial points regarding Mikulicz's disease Prognosis and relationship with lymphoproliferative diseases The long-term prognosis of MD is unknown.[link.springer.com]
  • Prognosis is usually good; rarely this condition may devolve into lymphoma, or could actually represent 'occult' lymphoma from the outset.[en.wikipedia.org]
  • 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]
  • With the exception of the eye changes the prognosis is favourable even without any treatment. — Mikulicz's syndrome, characterized by symmetrical enlargement of the lachrymal and salivary glands, may be caused by a variety of systemic diseases, such as[thieme-connect.com]

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]
  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • PURPOSE: 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]
  • 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]
  • 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]
  • Because the pathophysiology is poorly understood, the rationale of several surgical treatments is rather weak. Parotidectomy removes the diseased gland but begs the question of specific treatment.[emedicine.medscape.com]

Prevention

  • 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]
  • In most cases the cardinal symptoms do not occur simultaneously, which usually prevents early diagnosis, unless parotid gland biopsy shows typical Boeck granulomas.[thieme-connect.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