Aug 23, 2015 introduction the aim of this study was to clarify the effectiveness of various imaging modalities and characteristic imaging features in the screening of igg4related dacryoadenitis and sialadenitis igg4ds, and to show the differences in the imaging features between igg4ds and sjogrens syndrome ss. Mikulicz disease md has long been considered a manifestation of sjogrens syndrome. Mikulicz disease chronic dacryoadenitis and sialadenitis. Mikulicz s disease has been considered a part of primary sjogrens syndrome since morgans 1953 report. Dec 20, 2006 mikulicz s disease md has been included within the diagnosis of primary sjogrens syndrome ss, but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids, leading to the recovery of gland function. If you have problems viewing pdf files, download the latest version of adobe reader. Many terms such as igg4related systemic sclerosing. Kuttner tumor kt is also reported to be an igg 4 related disease. In this study, we examined the clinicopathologic and serologic findings in md 39 patients and kt 6 patients and. Immune responses in mikulicz disease differ from those in ss. This disease is rare in medical practice but is immediately recognizable.
In the seven decades since mikulicz described the disease entity which bears his name, there has been much confusion between mikulicz s disease and mikulicz s syndrome. Mikulicz s disease represents persistent enlargement of the lacrimal and salivary glands, and autoimmune pancreatitis is shown with diffuse pancreatic swelling. Igg4related chronic sclerosing dacryoadenitis external. Mikulicz s disease is a unique condition involving enlargement of the lacrimal and salivary. S ir, mikulicz s disease md represents a unique condition involving symmetrical enlargement of the lacrimal and salivary glands, the cause of which is unknown. As late as 1937 one author 1 divided herpes zoster into two distinct groups. Mikuliczs disease and sjogrens syndrome keio university. Were characteristic of mikulicz syndrome along with lympho epthleial islands. Aps 1, autoimmune polyendocrine syndrome type 1, autoimmune polyendocrinopathycandidiasisectodermal dystrophy apeced, hypoadrenocorticism with hypoparathyroidism and superficial moniliasis, autoimmune polyendocrinopathy syndrome type 1, polyglandular autoimmune syndrome type 1, pga 1, pgai, whitaker syndrome, type i polyglandular.
Females are affected more commonly than males and patients are typically in their middle years. Mikulicz s disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. Mikulicz disease or syndrome references in the icd10cm index to diseases and injuries. Mikulicz syndrome is a chronic disease in which the glandular tissue of the head and neck are excessively enlarged. This case report hypothesizes that the nonhodgkins lymphoma nhl, which in association with sjogrens syndrome and mikulicz disease in this patient comprised the mikulicz syndrome, may have transformed into his malignant largecell gastric lymphoma.
Mikulicz disease is defined clinically as bilateral painless swelling of the submandibular, sublingual, parotid, and lacrimal glands for the duration of at least 3 months. Case report mikulicz syndrome a cytological diagnosis in. In 1888, mikulicz1 reported before the verein fur wissenschaftliche heilkunde a case of bilateral, chronic, painless enlargement of the lacrimal glands with an associated enlargement of the salivary glands in a man 42 years of age. Recent developments in our understanding of gastric lymphoma. To characterize lacrimal gland function and lymphocyte infiltration in patients with mikulicz s disease md and sjogrens syndrome ss.
Igg4related diseaseigg4rd, formerly known as igg4related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and igg4secreting plasma cells, various degrees of fibrosis scarring and a usually prompt response to oral steroids. As time passed, this disease came to bear the name of mikulicz. Pdf mikulicz s syndrome is a chronic swelling of the lacrimal and major salivary glands usually associated with significantly decreased or lack of. Mikulicz syndrome definition, causes, symptoms, diagnosis. A new syndrome was identified recently, called igg4 syndrome.
While this can be a benign selflimiting condition, other complex systemic diseases, such as. The study showed that serum igg4 concentrations are elevated in patients with mikulicz s disease, but not in those with sjogrens. The association of herpes zoster with other diseases has always presented a fascinating study. The book examines how this development has changed the way. The condition is selflimiting and most often, the diagnosis is a clinical one. Effectiveness of imaging modalities for screening igg4. Mikulicz syndrome nord national organization for rare disorders. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. The probable systemic nature of mikuliczs disease and its. Chronic dacryoadenitis associated with enlargement of salivary glands causes include. Elevated igg4 concentrations in serum of patients with.
Mikulicz syndrome associated with a malignant large cell gastric. Clinicopathologic similarities between mikulicz disease. Mikulicz syndrome also known as igg4related dacryoadenitis and sialadenitis, dacryosialoadenopathy, mikulicz s disease, mikulicz radecki syndrome or mikulicz sjogren syndrome, is an immunoglobulin g4 igg4related disease characterized by inflammation of the lacrimal glands which produce tears, parotid glands, and submandibular glands two of the major salivary glands 1. Yamamoto et al listed criteria for the diagnosis of mikulicz s disease. References in the icd10cm index to diseases and injuries applicable to the clinical term mikulicz disease or syndrome mikulicz disease or syndrome k11. Previously, mikulicz s disease was often considered as a subtype of sjogrens syndrome ss.
Ibm, inflammatory myopathy, sporadic inclusion body myositis. The incidence of mikulicz disease and mikulicz syndrome is unknown but thought to be quite rare. Mikulicz s syndrome is defined as diffuse and bilateral enlargement of the salivary and lacrimal glands. American autoimmune related diseases association aarda. It is associated with other entities like tuberculosis, lupus. The literature records attempts to differentiate the two by means of biopsy, but histopathology, particularly in mikulicz.
Ihrler s, harrison jd oral surg oral med oral pathol oral radiol endod, 1003. It has been included within the diagnostic criteria for primary sjogrens syndrome ss due to their histological resemblance. Mikuliczs disease md has been included within the diagnosis of primary. Apparently, this was the first report of its kind given much attention in the literature, and in time the condition received the name mikulicz s disease. Mikulicz syndrome journal of oral and maxillofacial surgery. A medical eponym honours a scientists accomplishment in discovering a disease, symptom, anatomical part, procedure or principle. Mikulicz syndrome, fnac piyush anand1, hiremath s s2, thippeswamy m t r2.
The sialographic differentiation of mikuliczs disease and. Mikulicz s syndrome, as md rarely developed into lymphoma or leukaemia 12. Mikulicz s disease former, mikulicz disease former, mikulicz syndrome former, chronic dacryoadenitis and sialadenitis inclusion body myositis. Mikulicz syndrome nord national organization for rare.
Pdf mikuliczs syndrome is a chronic swelling of the lacrimal and major salivary glands usually associated with significantly decreased or lack. Mikulicz s disease was recently reported to be associated. There is no definitive curative treatment for either mikulicz or sjogren syndrom. Apr 02, 2021 free pdf download behcet syndrome the extended second edition of this important work provides an uptodate and comprehensive overview of behcets syndrome. A new conceptualization for mikuliczs disease as an igg4. Keywords lacrimal gland swelling, mikuliczs disease, salivary gland. In approximately 5170% of people with this disease, serum igg4 concentrations are elevated during an. Introduction immunoglobulin g4igg4related disease is a lymphoproliferative disorder associated with hyper igg4 gamma. Mikulicz disease sarcoidosis tuberculosis syphilis mumps.
Mikulicz s syndrome is a chronic swelling of the lacrimal and major salivary glands usually associated with significantly decreased or lack of lacrimation and xerostomia, accompanied by lymphocytic infiltration. The literature records attempts to differentiate the two by means of biopsy, but histopathology, particularly in mikulicz s disease, is uncertain. In a 12yearold boy with clinical and histologic features of mikulicz disease, systemic lupus erythematosus sle developed two years later. In the last few decades, the evolution of biomedical sciences has contributed to a better understanding of the pathogenesis of human disease, the description of new clinical entities, sorting out of disease subgroups, and development of targeted therapeutic interventions1. Mikulicz syndrome is a term used to denote any bilateral chronic enlargement of the lacrimal and salivary glands due to a specific disease process other than. It is associated with other entities like tuberculosis, lupus erythematosus, lymphoma, leukemia, etc. Mikulicz syndrome is a term used to denote any bilateral chronic enlargement of the lacrimal and salivary glands due to a. Igg4related mikulicz s disease is a multiorgan lymphoproliferative disease distinct from sjogrens syndrome. Mikulicz s syndrome, salivary gland swelling, extranodal lymphoid infiltrates, download full text pdf 1241 kb please cite this article as. Mikulicz syndrome associated with a malignant large cell. Differential diagnosis in mikuliczs syndrome, mikuliczs. It is an enlargement of salivary and lachrymal glands, which are drawn by xerostomia. Schaffer linked the case with obvious diseases, such as sarcoidosis and lymphoma, presenting these symptoms as mikulicz s syndrome and idiopathic cases as mikulicz s disease md in 1927.
Request pdf a case report of mikulicz syndrome mikulicz syndrome ms is a rare chronic condition characterized by the abnormal enlargement of glandular tissue in the head and neck. Analysis of igg fractions was performed in patients with mikulicz s disease in order to determine the differences between mikulicz s disease and sjogrens syndrome. Jul 12, 2009 it is responsive to glucocorticoid treatment. Mikulicz syndrome ms is a rare chronic condition characterized by the abnormal enlargement of glandular tissue in the head and neck. The lymphocytic infiltration and the relatively common clinical presentation has led to the acceptance of mikulicz s syndrome and sjogren syndrome to be considered as the same entity, and for many years the two terms have been used interchangeably. Mikulicz disease or syndrome icd10cm index to diseases. Mikuliczs disease and sjogrens syndrome request pdf. However, in the resent years the two syndromes have been dis.
In more recent times, the terms mikulicz s disease and mikulicz s syndrome were viewed as ambiguous and outdated by some sources. Clinicopathologic similarities between mikulicz disease and. Mikulicz syndrome is a chronic condition characterized by the abnormal enlargement of glands in the head and neck, including those near the ears parotids and those around the eyes lacrimal and mouth salivary. An ultrasound examination of the area may help to rule out other reasons for gland swelling. Liji thomas, md mikulicz syndrome is a chronic disease in which the glandular tissue of the head and neck are excessively enlarged.
May 19, 2014 endothelial cells in a hemorrhagic background. Clinical and pathological differences between mikuliczs disease. Mikulicz s disease md is a wellknown disorder characterized by enlarged lacrimal and parotid glands caused by infiltration with lymphocytes. Case report mikulicz syndrome a cytological diagnosis in an. Mikuliczs disease is a unique condition involving enlargement of the lacrimal and salivary glands, as is also seen in sjogrens syndrome. Immune responses in mikulicz disease differ from those in ss nature.
Both diseases are characterized with elevated igg4 concentrations in the serum and prominent infiltration by plasmacytes expressing igg4 in. Sep 20, 2017 igg4related dacryoadenitis and sialoadenitis formerly called mikulicz disease is an igg4related disease characterized by inflammation of the lacrimal glands which produce tears, parotid glands, and submandibular glands two of the major salivary glands. Diagnostic and management challenges in mikulicz syndrome. Original article from the new england journal of medicine mikulicz s syndrome with chronic lymphatic leukemia. Review igg4related mikuliczs disease is a multiorgan.
Mikuliczs disease and systemic igg4related plasmacytic. Deliverska e, stefanov l, hadjiev e, mihaylova z, karayasheva d. Article information, pdf download for mikuliczs disease. Thus aspiration cytology correlated with clinical features is an important tool for diagnosis of mikulicz syndrome. Beneficial effects of steroid therapy for mikuliczs. The tonsils and other glands in the soft tissue of the face and neck may also be involved. Mikuliczs syndrome with chronic lymphatic leukemia nejm. Terminology it may also be referred to by numerous other eponyms as well as the rather ve. Beneficial effects of steroid therapy for mikuliczs disease. The current data of md after the discovery of the hyper igg4related disease since the. Mikulicz disease md has long been considered a manifestation of sjogrens syndrome ss, but recent studies have proposed that md is a clinical entity in itself and is part of the spectrum of.
Jeffrey medeiros, in diagnostic surgical pathology of the head and neck second edition, 2009. Eponyms can be very helpful for professional communication, because they evoke characteristic. Clinical and pathological differences between mikuliczs. The authors are from sapporo medical university in hokkaido, japan.
Patients usually present with enlarged lacrimal and parotid glands. Autoimmune disorders are caused when the bodys natural defenses against foreign or invading organisms e. Mikulicz disease and syndrome jama otolaryngologyhead. These criteria can help to distinguish these patients from those with other conditions such as sjogrens syndrome. C, extensive symmetrical swelling of the palatal minor salivary glands.
Although the pathology is similar for md and ss, the difference in acinar cell apoptosis and function can explain clinical differences. Mikulicz syndrome is an autoimmune disease, considered a form of sjogrens syndrome. Methods thirtynine patients with igg4ds, 51 with ss and 36 with normal salivary. Mikuliczs syndrome, salivary gland swelling, extranodal lymphoid infiltrates. In 1888, mikulicz1 reported before the verein fur wissenschaftliche heilkunde a case of bilateral, chronic, painless enlargement of the lacrimal glands with an. Mikuliczs disease usually involves a single major salivary gland, but bilateral or multiple lesions may occur. Igg4related dacryoadenitis and sialadenitis genetic and. The exact cause of mikulicz syndrome is not known, although it is suspected to be an autoimmune disorder.
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