Mikulicz Syndrome Research Articles

Mikulicz's disease and systemic IgG4-related plasmacytic syndrome (SIPS)

Mikulicz's disease represents persistent enlargement of the lacrimal and salivary glands, and autoimmune pancreatitis is shown with diffuse pancreatic swelling. Both diseases are characterized with elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the glands. Clinical analyses were performed in 40 patients with systemic IgG4-related plasmacytic syndrome (SIPS) who consulted the doctors in Sapporo Medical University Hospital. Our patients were mainly middle-aged or elderly females. The average age was 58.9 years. The diagnosis was following ; 33 cases with Mikulicz's disease, 3 cases with Küttner's tumor, and 4 cases with IgG4-related dacryoadenitis. Slight dysfunction of lacrimal and salivary gland was observed in about 60% of them. Antinuclear antibodies were detected in only 15% of the cases with SIPS. Almost all, except one case, did not have anti-SS-A or anti-SS-B antibodies. Interestingly, hypocomplementemia was revealed in 30% of them. The complications of SIPS include autoimmune pancreatitis, tubulointerstitial nephritis, retroperitoneal fibrosis, prostatitis, and so on. SIPS is mainly treated by the administration of steroids. We started to prescribe much quantity of prednisolone to the patients with organ failure. The recurrence was admitted in the 3 patients for the followed 16 years. We present here the problems and prospects in SIPS.

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

To remove the confusion surrounding the terms "Mikulicz's disease" and "Mikulicz's syndrome" by discovering the nature of the disease that Mikulicz described. Investigation of the original 1892 publication by Mikulicz in the light of current knowledge. Mikulicz described bilateral painless swellings of the lacrymal, major salivary, and minor salivary glands that were present for 14 months without a reduction in salivary flow before the patient, a man of 42 years, died. A print of a microscopical field of one of the submandibular glands contains a uniform lymphocytic infiltrate of centrocyte-like cells typical of MALT lymphoma, which is strongly supported by the reported clinical features. Mikulicz published the first case report of a MALT lymphoma of salivary glands, and the terms "Mikulicz's disease" and "Mikulicz's syndrome" should no longer be used.

Jan Mikulicz-Radecki: one of the creators of world surgery

This paper presents the life and achievements of Professor Jan Mikulicz-Radecki and his contribution to European and world surgery. He was born in 1850. Four periods can be distinguished in his surgical career: (1) Vienna period, 1875-82, when he worked by the side of the great Theodor Billroth: he introduced a number of new diagnostic and operative techniques, aseptic and antiseptic procedures, published research papers. He made the first endoscope for examining the esophagus and stomach. (2) Cracow period, 1882-87, when he was head of the Department of Surgery: he inaugurated his work with a lecture in Polish, which started: Gentlemen, I have been accused that I do not know the Polish language--which is my mother tongue to me as well as to any of you. He published papers on the use of iodophorm for healing wounds, was the first surgeon who sutured a perforated gastric ulcer (1885) and invented pyloroplasty (1887), nowadays called Heinecke-Mikulicz pyloroplasty. (3) Konigsberg period, 1887-90: he improved the technique of gastric resection, worked on surgery for peptic ulcer and advocated aseptic and antiseptic procedures. (4) Wroclaw (Breslau) period, 1890- 1905: Mikulicz was appointed head of a newly founded Surgical Department. He rebuilt it and designed one of the largest and most modern operating theaters in Europe that time. He introduced silk gloves for operations for the first time in the world. In 1892 he described bilateral swelling of salivary and lacrimal glands, called Mikulicz's syndrome; in 1902 he published his experience with two-stage colon resection; and in 1904 he described ostitis fibrosa cystica juvenilis. Called "a king in the kingdom of surgeons", he was one of the fathers of the world's modern surgery; having a brilliant surgical mind, he was a pioneer of many new operations and an inventor of surgical tools, permanently assimilated in the world's surgery.

両側顎下腺腫ちょうをきたす疾患の病態

Diseases showing bilateral enlargement of the Submandibular glands were evaluated with respect to their clinical and histological features. It is well known that sialolithiasis is one of the most common diseases causing submandibular gland enlargement. Pleomorphic adenoma is also a common tumor of submandibular gland origin. These diseases usually cause unilateral swelling of the gland. This report describes several diseases affecting both glands, as follows: mumps, sialodochitis fibrinosa, Kuttner's tumor, Mikulicz syndrome, sialadenosis, sialolithiasis, malignant lymphoma and amyloidosis. Sialodochitis fibrinosa is charaterized by white jelly-like material obtained from Wharton's duct containing numerous eosinophils. Most cases of sialadenosis show parotid gland swellings, however, those associated with anorexia nervosa cause swellings predominantly in the submandibular gland. Malignant lymphoma is a rare tumor arising in the parotid and/or submandibular glands. Our cases resembled Kuttner's tumor or sialadenosis on palpation of the glands. Patients with amyloidosis were initially suspected of having Kuttner's tumor because very few patients visiting ENT clinics have bilateral gland swelllings caused by amyloidosis. Therefore, the differential diagnosis of bilateral submandibular enlargement is often difficult. In accordance with each case, careful examinations are needed using ultrasonograms, sialograms, RI scintigrams, CT scans, MRI and blood tests. When necessary, we add FNA or open biopsy.

Mikulicz syndrome associated with malignant large cell lymphoma of the stomach

Mikulicz syndrome associated with malignant large cell lymphoma of the stomach

Peripancreatic lymphoadenopathy and extrahepatic immunological manifestations in chronic hepatitis C.

The aim of the study was to determine the role of peripancreatic lymph node swelling in systemic immunological alterations during chronic hepatitis C (HC). The prospective study was carried out as a clinical study in a university hospital. Clinical, haematochemical and ultrasonographic findings in 182 patients were studied. Ultrasonography was performed by the same operator and the findings were evaluated blind without the operator knowing the clinical and haematochemical parameters. Hepatitis B virus (HBV) markers, anti-HCV antibodies, LKM1, cryoglobulinaemia, rheumatoid factor and anti-tissue antibodies were determined. Liver biopsy was carried out in 43 of the 182 patients. One or two pathological peripancreatic lymph nodes (PLNs) were present in 30 of the 182 patients and, of the 30, 28 were anti-HC positive. Only one patient in the non-PLN group was positive for anti-HCV, there being statistical significance (P < 0.0001) between the PLN and non-PLN groups. In HCV-positive patients, extrahepatic immunological manifestations were observed (cryoglobulinaemia; positivity to anti-smooth muscle, antinuclear and antimitochondrial antibodies; positivity to rheumatoid factor and LKM1). In five patients the presence of focal lymphocytic aggregates was detected by biopsy, whereas one patient presented typical ocular lesion of Mikulicz's syndrome. Our results may confirm the marked lymphotropism shown by the HC virus and indicate more complex immune system involvement, especially in view of the coexisting signs of immune system involvement related to the presence of intrahepatic cellular aggregates detected in our study. We believe that the peripancreatic adenopathy in chronic HCV hepatitis is an important diagnostic sign and may indicate an involvement of the C virus in the still unexplained extrahepatic immunological disorders.

SJÖGREN'S SYNDROME

Sjogren's syndrome is a chronic systemic inflammatory disorder characterized by the presence of dry eyes and dry mouth. Dryness in the eyes and mouth are secondary to diminished glandular production secondary to lymphocytic and plasma cell infiltration. Because of the prominent autoimmune features of this condition, Sjogren's syndrome has recently been referred to as an autoimmune exocrinopathy. 32 Although there is no universally accepted definition of Sjogren's syndrome, the diagnostic triad of keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth) associated with the presence of a connective tissue disorder is commonly employed. 7 Dry eyes may occur with or without lacrimal gland enlargement. Dry mouth may occur with or without salivary gland enlargement. Both primary and secondary forms of this disorder are recognized. Although primary Sjogren's syndrome may be a distinct disorder in the absence of another known connective tissue disease, the most frequent association of Sjogren's syndrome and a connective tissue disorder is with rheumatoid arthritis. Approximately one half of all patients with rheumatoid arthritis will present with Sjogren's syndrome whereas a less frequent association is seen with less common connective tissue disorders such as systemic lupus erythematosus or scleroderma. 30,49 It is important to distinguish these patients from patients that present with sicca complex, which includes only the presence of ocular and oral dryness. Because patients with primary sicca do not appear to have the systemic disease associations that are discussed in this article, they clearly would have a more favorable prognosis. Historically, features of Sjogren's syndrome have been recognized for over a decade. In 1892, Mikulicz reported the findings of bilateral parotid and lacrimal gland enlargement associated with significant round cell inflammation. 24 Although numerous reports appeared between 1883 and 1933 regarding patients with Mikulicz's syndrome, it was not until 1933 that Heinrich Sjogren described the syndrome that bears his name. 31 It is now generally accepted that Sjogren's syndrome and Mikulitz's disease are in fact variants of the same disorder. 25 It is important for the clinician to consider the diagnosis of Sjogren's syndrome in a patient who presents with suspected allergic disease. Patients with Sjogren's syndrome may present not only with dry eyes and dry mouth but may have prominent respiratory and ocular manifestations that are suspected to be of allergic origin. Because Sjogren's syndrome is suspected to affect 2 to 3 million individuals in the United States alone, 46 it is considered to be in fact the most common autoimmune disease process. Given its reasonably frequent occurrence and its potential for associated lymphoid malignancies, it is crucial that the clinician be aware of the manifestations of this disorder.

Benign lymphoepithelial lesion (Mikulicz's syndrome) of the submandibular glands in a four-year-old boy

Benign lymphoepithelial lesion (Mikulicz's syndrome) of the submandibular glands in a four-year-old boy

Benign Lymphoepithelial Lesion (Mikulicz's Syndrome) of the Submandibular Glands in a Four‐Year‐Old Boy

Benign Lymphoepithelial Lesion (Mikulicz's Syndrome) of the Submandibular Glands in a Four‐Year‐Old Boy

Mikulicz syndrome and disease: 2 case reports highlighting the difference

We present 2 case reports highlighting the essential differences between Mikulicz syndrome and Mukulicz disease. A review of the literature appears to show some confusion over the terminology and we therefore also present an historical account of the evolution of the two terms and review their relative merits and relationship with other disorders. In conclusion, we would not advocate the use of the term Mikulicz syndrome and suggest that Mikulicz disease be replaced by the title 'Benign Lymphoepithelial Lesion'.

ミクリッツ症候群をきたした限局性アミロイドーシスの一症例

ミクリッツ症候群をきたした限局性アミロイドーシスの一症例

A case of sarcoidosis initially presented Mikulicz syndrome, then complicated with hydrocephalus during the following steroid therapy

A case of sarcoidosis initially presented Mikulicz syndrome, then complicated with hydrocephalus during the following steroid therapy

Mikulicz syndrome

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 Mikulicz disease (benign lymphoepithelial lesion). The purposes of this article are to describe the differential diagnosis of Mikulicz syndrome, illustrate it by a case arising from malignant lymphoma, and make an attempt to place Mikulicz, his syndrome, and his disease in historical perspective, thereby shedding light on the confusion which has surrounded these terms in recent literature.

Manifestations of parotid gland enlargement: radiographic, pathologic, and clinical correlations. Part II: The diseases of Mikulicz syndrome.

Manifestations of parotid gland enlargement: radiographic, pathologic, and clinical correlations. Part II: The diseases of Mikulicz syndrome.

Mikulicz's disease and Mikulicz's syndrome.

The pathogenesis, clinical manifestations, and historical development of Mikulicz's disease and Mikulicz's syndrome are given. It is recommended that the term "Mikulicz's syndrome" be dropped to avoid confusion with the rare and well defined entity "Mikulicz's disease."

Asymptomatic Parotid Enlargement: Probable Diabetic Origin

To the Editor.— The appearance of asymptomatic, bilateral parotid enlargement is widely recognized as a relatively frequent clinical occurrence. It has been noted in Mikulicz syndrome associated with sarcoidosis, in Reiter syndrome, and in several other disorders. However, it is not generally appreciated that this interesting physical finding can also be associated with diabetes mellitus. Report of a Case.— A 50-year-old woman's condition was first diagnosed as adult-onset diabetes mellitus at age 46. After evaluation at another hospital, she was placed on an American Diabetes Association diet and a daily dose of approximately 100 mg of tolbutamide. During the succeeding four years, the patient demonstrated poor diabetic control on numerous occasions and was eventually switched to tolazamide, 500 mg daily. On the day of admission to our hospital, the patient was complaining of dizziness, weakness, and faintness. She gave a history of diabetes, and a blood glucose level determined at

Granulocytic Sarcoma as the Presenting Manifestation of Acute Leukemia in Childhood

This report describes six cases of acute leukemia in childhood manifested initially by granulocytic sarcoma. Solid tumor was associated with acute monocytic leukemia in one patient and with acute myelogenous leukemia in other patients. In two patients, generalized lymphadenopathy was the predominant feature preceding other signs and symptoms of acute leukemia. One case revealed the clinical course of smoldering acute leukemia characterized by insidious onset and a small number of myeloblasts in the bone marrow. One case showed Mikulicz's syndrome preceding the systemic manifestation of leukemia, and the peripheral blood was normal at the time of admission. In another patient with an orbital tumor, three months after the biopsy which was reported to be reticulum cell sarcoma, blood and marrow were typical of acute leukemia. In this case, peripheral blood picture was normal at the time of tumor formation, but marrow examination was not performed. The last case was unusual in that myeloblastoma preceded the blood and marrow evidence of acute leukemia by 38 months. Review of literature revealed 6 well-documented cases of granulocytic sarcoma preceding the marrow evidence of leukemia. The importance of cytological examination by Peroxidase reaction and May-Giemsa staining on imprints prepared from biopsy specimen together with histological examination in differentiating acute leukemia from reticulum cell sarcoma was stressed. Chloromas are green tumor masses composed of primitive white blood cells, usually associated with acute myelocytic or myelomonocytic leukemia, occurring more often in childhood than in adults. Myeloblastoma—tumorous masses histologically identical to chloroma but without greenish pigmentation—is often used as a synonym. But the term granulocytic sarcoma may be more appropriate to describe these tumors (Rappaport, 1966). Occasionally, tumor formation may be the earliest manifestation preceding other symptoms, signs and bone marrow evidence of acute leukemia.

Iodide intoxication: Report of a case

Iodide sensitivity or intoxication is infrequently encountered in clinical procedure. The following is a brief report of a case of iodide sensitivity, including signs, symptoms, and treatment. The similarity of iodide intoxication and other clinical entities such as Mikulicz's disease, Mikulicz's syndrome, Sjögren's syndrome, malignant lymphomas, and uveoparotitis is mentioned. The importance of taking a thorough history is emphasized.

Differential Diagnosis in Mikulicz's Syndrome, Mikulicz's Disease, and Similar Disease Entities

Differential Diagnosis in Mikulicz's Syndrome, Mikulicz's Disease, and Similar Disease Entities

Mikulicz's Syndrome due to Sarcoidosis

Mikulicz's Syndrome due to Sarcoidosis