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.
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 . Such designations are now considered outdated and ambiguous.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   .
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  .
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.
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  .
- Muscle Rigidity
These drugs have the ability to prevent muscle spasms and were first developed to prevent muscle rigidity associated with seizures. Thus, patients who look up Klonopin are surprised to see that it was first approved for children with seizures. [dry.org]
He developed polyuria and polydipsia after initiation of steroid therapy. Plasma and urine osmolality were suggestive of central diabetes insipidus (Table I). His serum Anti-Diuretic Hormone (ADH) was not estimated. [journals.viamedica.pl]
Jaw & Teeth
- 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]
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]
- 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]
Face, Head & Neck
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  .
- 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]
A critical evaluation of enzyme immunoassays for detection of antinuclear autoantibodies of defined specificities. I. Precision, sensitivity, and specificity. Arthritis Rheum 42:455. 23. Vivino, F. B., I. Gala, and G. A. Hermann. 2002. [dry.org]
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]
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- 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.
- Lemp M. Report of the National Eye Institute/industry workshop on clinical trials in dry eyes. CLAO J. 1995;21:221-232.
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- 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.
- 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.
- 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.
- Tsubota K, Fujita H, Tsuzaka K, al. e. Mikulicz’s disease and Sjögren’s syndrome. Invest Ophthalmol Vis ScI. 2000;41:1666-73.