Sir, mikuliczs disease md represents a unique condition involving symmetrical enlargement of the lacrimal and salivary. Although considered a rare disease, it is endemic in lowincome countries where hygienic conditions are poor. Igg protein is comprised of molecules of 4 subclasses designated igg1 through igg4. Rare diseases are usually genetic, chronic and incurable disorders with a relatively low incidence. Previously, mikuliczs disease was often considered as a subtype of sjogrens syndrome ss. 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. Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. Of total igg, approximately 65% is igg1, 25% is igg2, 6% is igg3, and 4% is. Surgical management of crohns disease aaron winnick, m. While this can be a benign selflimiting condition, other complex systemic diseases, such as sarcoidosis, may represent other underlying. The tonsils and other glands in the soft tissue of the face and neck may also be involved. Sjogrens showgrins syndrome ss is a disorder of your immune system identified by its two most common symptoms dry eyes and a dry mouth. Therapy, tsuboi and colleagues demonstrated that regulatory t treg celland t helper 2 th2 cellderived. Mikuliczs disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions.
Each subclass contains molecules with a structurally unique gamma heavy chain. The type k3 capsule of klebsiella rhinoscleromatis is a virulence factor not involved in mikulicz cells formation. Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs. Some diseases are acute, producing severe symptoms that terminate after a short time, e. Lip biopsy in mikuliczs disease phenotype igg4related disease. Recent reports suggest that igg4rd is a major cause of mikulicz disease5,16. Extrasalivary lymphoid abnormalities in sjogrens syndrome. Pdf mikuliczs disease and its extraglandular lesions.
Capsule polysaccharide cps is a prominent virulence factor in. Mikulicz disease chronic dacryoadenitis and sialadenitis. Mikuliczs disease of the lacrimal gland jama ophthalmology. Dec 01, 2008 rhinoscleroma rs is a chronic, progressive, granulomatous infectious disease of the upper respiratory tract. It was recognised as a unified entity only 10 years ago. 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. Please feel free to add the profile of anyone living with or who lived with sjogrens syndrome. In some cases, it also affects other glands or organs. Full text is available as a scanned copy of the original print version. Mikulicz disease is characterized clinically by bilateral, painless, symmetric swelling of the lacrimal, parotid, and submandibular glands. American autoimmune related diseases association aarda.
Kings county hospital center suny downstate medical center september 29, 2006. Igg4related disease has been described in virtually every organ system. Mikuliczs disease md has been included within the diagnosis of primary sjogrens syndrome ss, but represents a unique condition invo. Pdf a case of mikuliczs disease complicated by autoimmune. A hallmark of this pathology is the appearance of atypical foamy monocytes called mikulicz cells. A case report of mikulicz syndrome request pdf researchgate. 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. It was first reported in europe, but it is now rarely diagnosed on that continent. Immunoglobulin g4 related disease igg4rd is a rare systemic fibroinflammatory disorder orpha284264. Type 1 autoimmune pancreatitis is the most frequent manifestation of igg4rd. A novel murine model of rhinoscleroma identifies mikulicz.
Mikulicz angle academic dictionaries and encyclopedias. Rhinoscleroma rs is a chronic, progressive, granulomatous infectious disease of the upper respiratory tract. 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. Malignant lymphoma of parotid associated with mikulicz. Terminology it may also be referred to by numerous other eponymous names as well as the c. Igg4related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. The condition is selflimiting and most often, the diagnosis is a clinical one. The most abundant immunoglobulin in human serum is immunoglobulin g igg approximately 80% of the total. Management of igg4related disease the lancet rheumatology. Mikulicz disease mikulicz disease disease, mikulicz disease, mikulicz francais. Mikuliczs disease of the lacrimal gland is a rare disease of unknown etiology. Pdf clinical and pathological differences between mikulicz. However, new data from japan suggest it may be a different disease with overlapping clinical features. Differential diagnosis in mikuliczs syndrome, mikuliczs disease.
Mikuliczs syndrome definition of mikuliczs syndrome by. Clinical and pathological differences between mikuliczs disease. Recently, it has been considered an igg4related disorder. Immunoglobulin g4related disease igg4rd is a systemic fibroinflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. Igg4rd mostly has been described in adults, hence it is generally unknown among pediatricians. B antibodies, but frequently have elevated serum igg4 levels.
Mikulicz disease article about mikulicz disease by the. Sjogren syndrome sjs, ss is a longterm autoimmune disease that affects the bodys moistureproducing glands. Immunoglobulin g4related disease with features of mikuliczs disease and autoimmune pancreatitis which firstly presented as asymptomatic lymphade. Several organ systems can be involved and encompasses many previous and newly described diseases such as type1 autoimmune pancreatitis. In the disease prevention category, there are healthlinkbc file topics about animal and insect borne diseases, food and water borne diseases, infectious diseases, diseases related to travel, as well as immunizations available in british columbia to protect against disease. The disease has strong organ predilections, and many of the clinical presentations of disease are increasingly. Mikulicz syndrome nord national organization for rare disorders. Mikuliczs disease is a unique condition involving enlargement of the lacrimal and salivary glands, as is also seen in sjogrens syndrome. The patient re turned december 26th, 1924, for com plete removal, as the mass interfered with the vision. Igg4 related renal disease, tubulointerstitial nephritis tin. However, igg4rd can affect any organ such as salivary glands.
Mikulicz syndrome ms is a rare chronic condition characterized by the abnormal enlargement of glandular tissue in the head and neck. Mikuliczs disease and sjogrens syndrome iovs arvo journals. However, the pathogenesis of rhinoscleroma remains poorly investigated. Mikuliczs disease mikuliczs disease md long has been considered a manifestation of sjogrens. Mikulicz disease definition of mikulicz disease by the. The proliferative stage is characterized by the appearance of mikulicz cells, a hallmark of this disease. Get a printable copy pdf file of the complete article 532k, or click on a page image below to browse page by page. Mikulicz syndrome associated with a malignant large cell gastric. Mar 17, 2010 patients with mikuliczs disease lack anti. It is found in a variety of diseases, including leukemia, tuberculosis, and sarcoidosis. If untreated, the disease can lead to fibrosis and irreversible organ damage. Case report delayed diagnose and successful radiation.
Patients usually present with enlarged lacrimal and parotid glands. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Igg4related disease was unrecognised as a unified disease entity until this century, yet in a short period of time the disease has been appreciated to have a worldwide distribution, and its clinical, pathological, and radiological features have been described in considerable detail. Diagnosis and classification of autoimmune pancreatitis. Dentalcraniofacial manifestation and treatment of rare diseases. Download fulltext pdf a case of mikuliczs disease complicated by autoimmune pancreatitis, in which impaired glucose tolerance was improved by glucocorticoid treatment. Jan 25, 20 the hallmarks of igg4related disease igg4rd are lymphoplasmacytic tissue infiltration with a predominance of igg4positive plasma cells, accompanied by fibrosis, obliterative phlebitis, dacryoadenitis, and elevated levels of igg4. Johann mikulicz1 first described, in 1888, a chronic selflimited.
Developments in the diagnosis and management of rare diseases have been relatively slow due to a. Developments in the diagnosis and management of rare. Mikulicz disease, kuttner tumor, inflammatory orbital pseudotumor. The purpose of the present study was to examine 21 patients 10 men, 11 women. Mikulicz syndrome nord national organization for rare. Five cases have been collected which show evidence at the same site of both mikulicz disease and a malignant lymphoma. Ormond syndrome is a condition characterized by the proliferation of fibrous tissue in the retroperitoneum5. To characterize lacrimal gland function and lymphocyte infiltration in patients with mikuliczs disease md and sjogrens syndrome ss. Year authors ref evidencescontents 1892 mikulicz j.
Igg subclass 4related disease is a recently recognized syndrome of unknown etiology most often occurring in middleaged and older men. There is much confusion in the literature regarding the differences between mikulicz disease and mikulicz syndrome. Keratoconjunctivitis sicca, xerostomy, arthritis sjogren. Four specimens of this disease, found in the files of the eye pathology department of the massachusetts eye and ear infirmary since 1896, are reported. Benign lymphoepithelial lesion mikulicz disease is generally regarded as an inflammatory disorder of unknown aetiology, characterized by epimyoepithelial islands, and unrelated to malignant lymphoma. Beneficial effects of steroid therapy for mikuliczs disease. This disorder, as first described by mikulicz, consists of a chronic symmetrical enlargement of the lacrimal and salivary glands. Specifically, mikuliczs disease is defined by a persistent and symmetrical swelling of the lacrimal and salivary glands together with elevated serum concentration of igg4. Get a printable copy pdf file of the complete article 245k, or click on a page image below to browse page by page. This multiorgan immunemediated condition links many disorders previously regarded as isolated, singleorgan diseases without any known underlying systemic condition. At this time, decem ber 26th, 1924, an attempt was made to remove the entire tumor growth but no effort was made to take the sur mikulicz disease r o u n d i n g tissue. In more recent times, the terms mikulicz s disease and mikulicz s syndrome were viewed as ambiguous and outdated by some sources. Idiopathic retroperitoneal fibrosis chronic sclerosing aortitisperiaortitis riedels thyroiditis igg4 related interstitial pneumonitis and pulmonary inflammatory pseudotumors.
Vagotomy and heinekemikulicz pyloroplasty in the treatment of pyloric stenosis. Clinical and pathological differences between mikuliczs disease and sjogrens syndrome article pdf available in rheumatology 442. Igg4related dacryoadenitis and sialadenitis rare disease. It is a longstanding disease that begins in the nasal mucosa and extends to other respiratory tract organs, producing airway obstruction and sometimes threatens the patient life.
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