Mikulicz Syndrome Research Articles

Thoracic manifestations of IgG4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons per year. The disease can affect virtually any organ and is characterized by unifying histopathological findings. Recently, four subgroups of patients have been characterized: hepatobiliary, head and neck, Mikulicz syndrome and retroperitoneal fibrosis, who illustrate the mainly abdominal and ENT tropism of the disease. Yet, thoracic involvement is not uncommon. It can be detected in up to 30% of patients with systemic IgG4-RD and is the exclusive manifestation of the disease in about 10% of cases. Clinical symptoms are nonspecific and may include dyspnoea, cough or chest pain. Chest CT findings are heterogeneous and primarily include peribronchovascular thickening, nodules, ground-glass opacities and lymphadenopathy. There is no specific diagnostic test for IgG4-RD thoracic involvement, which may mimic malignancy or vasculitis. Therefore, a cautious approach is needed to make an accurate diagnosis: a search for extra-thoracic manifestations, elevated serum IgG4 levels, circulating levels of plasmablasts and pathologic evidence of disease is warranted. Although very suggestive, neither the presence of a polyclonal IgG4 lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis are sufficient to confirm the histological diagnosis. Steroids are recommended as first-line therapy. Rituximab or disease-modifying antirheumatic drugs may be used in relapsed or rare cases of steroid-refractory disease. In this review, we summarize current knowledge regarding the pathophysiology, epidemiology, diagnostic modalities (clinical-biological-imaging-histopathology) and treatment of IgG4-RD thoracic involvement.

Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study

BackgroundOral corticosteroid remains the first-line treatment of IgG4-related ophthalmic disease, but steroid-dependence is common and serious. Factors associated with steroid dependence and relapse have to be further explored.Study populationA city-wide,...

Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: a retrospective study of 127 Chinese patients

Background and aimsImmunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse.MethodsWe collected baseline data of eligible patients with IgG4-RD and analyzed clinical features by interview and review of medical records. The patients who received glucocorticoids (GC) therapy with at least 3 months follow-up were used to characterize the therapeutic response and identify risk factors for relapse.ResultTotally 127 IgG4-RD patients, including 92 males and 35 females, were enrolled in the present study. The median age of onset was 63.0 years, ranging from 23 to 86. The pancreas, bile duct and lymph nodes were the most frequently involved organs. The serum IgG4 level was elevated in 94.5% of the patients and was correlated with the number of organs involved. Patients classified as head and neck limited group were more likely to be female. Compared to Mikulicz syndrome and systemic involvement group, pancreato-hepatobiliary group had higher aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, bilirubin and lower IgG4 level. Mikulicz syndrome and systemic involvement group had the highest IgG4-RD RI score, IgG level. Among 92 patients who received medical therapy with at least 3 months follow-up, 76 received GC alone or in combination with immunomodulator (IM) and 16 patients did not take GC. 74 out of the 76 patients (97.3%) achieved remission, with 59 of them remained in remission and 15 of them relapsed. Whereas 16 patients did not take GC, among them, 6 patients achieved remission with one relapsed. On multivariate analysis, higher initial score of ACR/EULAR IgG4-RD Classification Criteria and GC withdrawal were independent predictors for relapse.ConclusionFour phenotypes of IgG4-RD showed different demographic and serological features. GC + IM therapy was safe and effective and might protect patients from relapse. The independent risk factors of relapse were GC withdrawal and higher score of ACR/EULAR IgG4-RD Classification Criteria.

Significance of high serum IgG4 in complete or non-full-fledged IgG4-related disease-a retrospective investigation of 845 patients and its clinical relevance.

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized heterogeneous, subacute, and usually silent autoimmune disease involving many organs with protean manifestations. However, high IgG4 in serum is not necessarily indicating an IgG4-RD. The aims of this study were to investigate the clinical relevance of high serum IgG4 level in IgG4-RD or non IgG4-RD patients, and to see if IgG4-RD in Taiwan differs from that in other parts of the world. Eight hundred forty-five patients with high IgG4 were retrospectively reviewed from January 2002 to May 2020 in Taipei Veteran General Hospital. Two hundred sixty-seven patients fulfilled IgG4-RD criteria and were categorized into pancreato-hepato-biliary disease, retroperitoneal fibrosis and/or aortitis, head/neck-limited disease, classic Mikulicz syndrome with systemic involvement, CNS-limited disease, sclerosing vasculitis, skin-limited disease, and sensorineural hearing disease. These manifestations were correlated to smoking, atopy, hyper-IgE/eosinophilia, aging, malignancies, and hypocomplementemia. Five hundred seventy-eight patients were not fulfilling the criteria but were also analyzed for the prevalence of allergy, malignancy, connective tissue diseases, lung diseases, and infections. In IgG4-RD patients, 124 (46.4%) smoked. Top 4 clinical subtypes included Mikulicz syndrome with systemic involvement (33.3%), pancreato-hepatobiliary disease (31.4%), head/neck disease (19.4%), and retroperitoneal fibrosis/aortitis (12.7%). Top 4 co-morbid conditions included high serum IgE/eosinophilia (46.2%), hypocomplementemia (34%), malignancies (13.4%), and allergy (13.4%). Pancreato-biliary disease was associated with high IgE/eosinophilia (r2 = 0.380, P = 0.025) and malignancy (r2 = 0.211, P = 0.027), Miculicz syndrome with allergy (r2 = 0.396, P < 0.01) and high IgE/eosinophil (r2 = 0.396, P < 0.01), CNS diseases (r2 = 0.973, P = 0.035) and sclerosing vasculitis (r2 = 1, P < 0.01) with advanced age respectively, with the latter being also related to atopy and high IgE/eosinophilia (r2 = 1, p < 0.01). Smoking may precipitate IgG4-RD. IgG4-RD with pancreato-hepatobiliary disease is closely related to allergy and neoplasm, and those with Mikulicz syndrome may result from atopy. Elderly IgG4-RD patients tend to develop CNS pathology parallel to advancing of age. The disease may probably be originated from an unknown mechanism that may sporadically evolve into malignancies.

Ophthalmic Manifestations of Immunoglobulin G4-Related Disease (IgG4-RD)

Immunoglobulin-G4 related disease (IgG4-RD) is an inflammatory disorder of unknown etiology that comprises a wide spectrum of presentation, involving nearly all the bodily systems. A number of clinical conditions which were previously identified as distinct entities, such as autoimmune pancreatitis, Reidel thyroiditis, and Mikulicz syndrome, have now come within the purview of this disease. The clinical expression of IgG4-RD varies depending upon the organ involved. The authors in this article aim to highlight the various ophthalmic manifestations of IgG4-RD.

MON-264 Pleotropic Clinical Presentation in Two Brazilians Patients with Confirmed IgG4-Related Hypophysitis

Background: Hypophysitis is a rare condition characterized by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement with mass effect symptoms. IgG4-related hypophysitis can occur alone or as part of a multiorgan disease. Treatment with glucocorticoids is effective in 97% of the cases in reducing pituitary mass. Clinical cases: Case #1. A 56-yrs man with previous diagnosis of Mikulicz syndrome was referred to our service with fatigue and erectile dysfunction. Laboratory evaluation revealed hypogonadotropic hypogonadism, hyperprolactinemia (PRL=108 ng/mL) and central hypothyroidism. Sellar MRI depicted a pituitary mass with pituitary stalk thickening and a homogeneous uptake of gadolinium. During clinical follow-up, he also presented retroperitoneal fibrosis and IgG4-related disease was confirmed by serum IgG4 elevation and a pathological review of the previous salivary gland biopsy.Prednisone 80 mg/d treatment was initiated, with recovery of the thyrotrophic axis, reduction of PRL levels and significant reduction of the pituitary lesion. Due to maintenance of inflammatory activity and worsening of renal function, azathioprine therapy was associated, with subsequent inclusion of rituximab. Case #2. A 16-yrs boy was referred to our service presenting severe headache, bilateral visual deficit, right eyelid ptosis, hyposmia, polyuria and polydipsia. Cranial MRI depicted an extensive skull base mass involving pituitary gland, optic nerves, cavernous sinuses, olfactory bulb and clivus). Hormonal evaluation confirmed normoprolactinemia, hypogonadotropic hypogonadism and diabetes insipidus. Biopsy of the lesion revealed meningeal inflammation with immunohistochemistry suggesting IgG4-related sclerosing disease. No other organs were affected. An important lesion reduction and gonadotropic axis recovery occurred after 40 days of prednisone 60mg/d. After the drug withdrawal, methotrexate was introduced. However, after three years, headache and hyposmia recurred. A new MRI revealed increase of lesion and mycophenolate and rituximab were initiated, with clinical improvement without recurrences over time. Conclusion: Although a rare disease, IgG4-related disease should be included in the differential diagnosis amongst pituitary masses, with or without other affected organs. Immunosuppression with corticosteroids is the first treatment choice and other alternatives must be used in case of persistence of disease activity or relapse. These are very few Brazilian patients reported with IgG4 related disease. We described two cases with IgG4-related hypophysitis: one young patient, without involvement of other organs and another of middle age with systemic involvement, reinforcing the pleotropic clinical picture. Both required rituximab therapy due to disease progression.

PB1685 RELAPSE OF ACUTE LYMPHOBLASTIC LEUKEMIA MIMICKING MIKULICZ SYNDROME DURING BLINATUMUMAB TREATMENT: A CASE REPORT

Background:Mikulicz syndrome is a rare immune mediated condition characterized by tumor‐like swelling, lymphoplasmacytic infiltrate and fibrosis of salivary and lacrimal glands of poorly understood etiology. Its clinical presentation in the course of treating acute leukemia can be hard to differentiate from progression of the disease. We present a challenging diagnostic case of extramedullary acute lymphoblastic leukemia (ALL) relapse in the course of blinatumumab.Aims:Case report that aims to remember that AAL relapse can mimic any clinical picture.Methods:A 42‐year‐old woman was diagnosed in May 2017 with acute pro‐B lymphoblastic leukemia Ph negative, EGIL B‐II. Treatment with intensive multiagent chemotherapy according to PETHEMA high risk group was started. After induction, patient achieved complete response (CR) with negative minimal residual disease (MRD). Four additional courses of chemotherapy were completed following the PETHEMA high risk schedule. First relapse occurred and salvage therapy with Fludarabine, Ara‐C and Idarubicin was started achieving 2nd CR. Unfortunately, the patient relapsed three months later in the bone marrow and blinatumumab was decided. Two cycles were administered without neurological toxicities achieving CR with MRD of 0.31% and 0.11% after 1st and 2nd cycles, respectively. On 26th day of the 3rd cycle, acute swelling of both submandibular glands, swelling of right parotid gland and right monocular diplopia were observed. Cervical and cranial magnetic resonance was performed (Image 1) showing significant enlargement of the right lacrimal gland and bilateral submandibular glands. Bone marrow aspiration (BMA) showed CR with improved MRD of 0.02%. Cytological and cytometric cerebrospinal fluid analysis did not find ALL infiltration. With no evidence of disease progression and given the characteristics of the clinical and radiological picture, Mikulicz Syndrome was raised as a probable diagnostic possibility. However, a submandibular glandular tru‐cut biopsy showed glandular infiltration by ALL cells. One week later, a body PET/CT found significant metabolic uptake activity in the glands, regional lymph nodes and extensive bone involvement. Then, a new BMA (performed 10 days after de previous one) demonstrated ALL massive medullar relapse.Results:Relapsed/refractory (R/R) ALL is a bad condition with poor survival. Isolated extramedullary ALL relapse occurs in up to 6% of ALL according to literature and is often hard to demonstrate. In our case, the picture started as neck and head glandular swelling and diplopia. In the absence of medullar disease, Mikulicz Syndrome was suspected. Mikulicz Syndrome is a rare immune chronic benign condition characterized of swelling of neck and head glands where diplopia is often the first clinical sign. It has been related with several diseases including sarcoidosis, lymphoma and acute lymphoblastic leukemia. In this scenario, a biopsy of the involved glands is strongly recommended to rule out leukemia progression. In our case, tru‐cut biopsy of one of the involved glands demonstrated AAL extramedullary relapse despite absence of medullar involvement. Unfortunately, massive medullar relapse was confirmed a week later.Summary/Conclusion:Mikulicz syndrome is a diagnostic challenge for clinicians. In the setting of patients with ALL under blinatumumab treatment, clinicians should rule out ALL relapse before confirming this rare condition.image

Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts

ObjectiveIgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes.MethodsWe...

DON’T LET THE DRY MOUTH FOOL YOU

SESSION TITLE: Signs and Symptoms of Chest Disease SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Sarcoidosis is a well-known though poorly understood disease process with a multitude of manifestations. Clinicians should be aware of the diverse manifestations, as any evidence of active sarcoidosis requires consideration for treatment. We present a case of Mikulicz syndrome, a rare presentation of sarcoidosis in which the parotid and lacrimal glands are infiltrated by granulomas with resulting dry mouth, dry eyes and occasionally recurrent fevers. CASE PRESENTATION: The patient is a thirty-seven-year-old African-American female with a history of pulmonary and dermatologic sarcoidosis. She was diagnosed after a skin biopsy revealed noncaseating granulomas in the setting of chronic dyspnea with bilateral hilar lymphadenopathy. Systemic glucocorticoids were initiated with improvement in her dyspnea and skin lesions. Over the next several years, she did not tolerate weaning attempts and experienced frequent flares requiring escalating doses of methotrexate and glucocorticoids that led to significant side effects to include diabetes, obesity and bilateral hip avascular necrosis. Ultimately, her dyspnea and cough resolved on methotrexate and her skin lesions improved with a combination of intralesional triamcinolone injections and hydroxychloroquine with successful weaning off of her steroids. However, in subsequent visits, she reported swelling in her jaw along with a dry mouth with no recurrent skin lesions or dyspnea. On exam, enlargement of the parotid and lacrimal glands was recognized as Mikulicz syndrome. As a result, adalimumab was initiated with resolution of her symptoms. DISCUSSION: Mikulicz syndrome is a rare presentation of sarcoidosis, occurring in only 2% of patients with systemic sarcoidosis. It can easily be overlooked or attributed to a different disease process such as Sjogren’s syndrome, IgG4 related disease, lymphoma, vasculitis and HIV. However, it has been associated with significant symptoms affecting quality of life and most importantly represents active disease that requires consideration of treatment. In this case, the early recognition of Mikulicz syndrome led to appropriate treatment with a TNF-alpha antagonist that otherwise may not have been started as her skin and pulmonary sarcoid was relatively quiescent. CONCLUSIONS: Sarcoidosis can present with symptoms in nearly any organ system with significant impact on treatment course and quality of life. Providers who routinely treat sarcoid should be aware of the diverse manifestations of the disease, including the relatively rare presentations like Mikulicz syndrome as they may alter treatment strategy. Reference #1: Ungprasert, Patompong, Cynthia S. Crowson, and Eric L. Matteson. "Clinical characteristics of parotid gland sarcoidosis: a population based study.” JAMA otolaryngology--head & neck surgery 142.5 (2016): 503. Reference #2: Rao, Dinesh, et al. "A Case Report of Mikulicz Syndrome.” Journal of radiology case reports 11.7 (2017): 1. DISCLOSURES: No relevant relationships by Arthur Holtzclaw, source=Web Response No relevant relationships by John Sherner, source=Web Response

Diagnostic and Management Challenges in Mikulicz Syndrome

ABSTRACT Mikulicz disease (MD) is a disorder characterized by multiple lymphoepithelial lesions involving lacrimal and salivary glands. It is an uncommon condition, and very few cases are reported in the literature. It is a variant of Sjögren syndrome (SS) with patients presenting with clinical features over a wide range of spectrum. It is important to differentiate on which side of the disease spectrum the patient is presenting, i.e., either primary SS or secondary SS, because management can vary. The purpose of mentioning these two cases and its importance lie in the fact that the patients presented at two ends of the spectrum of SS – the first patient belonging to secondary SS and the second patient belonging to primary SS – which changes the line management for both patients, conservative and surgical respectively. The purpose of reporting these two cases is to emphasize the importance of identifying disease symptoms and signs early, which can help stratify the patient as a variant of primary SS or secondary SS and thus decide on the appropriate line of management, saving precious time and discomfort to the patient. Here we are mentioning two case reports of MD belonging to diagonally opposite spectrums of SS and their management with successful remission. How to cite this article Bhattacharjee A, Uddin S, Prakash R, Rathor A, Kalita S. Diagnostic and Management Challenges in Mikulicz Syndrome. Int J Head Neck Surg 2015;6(4): 139-145.

Igg4-Related Disorder and Endocrine Diseases: A Review of the Literature

IgG4-related disorders (IgG4-RD) are a new entity of multi-organ disorders that share common fibro-inflammatory histopathologic characteristics and elevated IgG4 levels in serum and tissue. Autoimmune pancreatitis and Mikulicz syndrome are major entities, however many syndromes are part of IgG4-RD including eosinophilic angiocentric fibrosis, fibrosing mediastinitis, hypertrophic pachymeningitis, inflammatory pseudotumour, multifocal fibrosclerosis (commonly affecting the orbits, thyroid gland, retroperitoneum, mediastinum, and other tissues and organs), periaortitis and peri-arteritis, inflammatory aortic aneurysm, retroperitoneal fibrosis (Ormond’s disease), Riedel’s thyroiditis, and sclerosing mesenteritis. In this review we detail the link between IgG4-RD and endocrine disorders and then examine the link between diabetes and IgG4-RD. We study the increased risk of IgG4-RD after using dulaglutide. We concluded that IgG4-RD should be suspected in patients with progressive thyroiditis with mass effect and very high antibody titers. The relationship between IgG4-RD and diabetes is still controversial.

Unusual Multiorgan Immunoglobulin G4 (IgG4) Inflammation: Autoimmune Pancreatitis, Mikulicz Syndrome, and IgG4 Mastitis

Autoimmune pancreatitis (AIP) type 1 is commonly associated with simultaneous involvement of extrapancreatic organs. Sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, Sjögren syndrome, and other extrapancreatic lesions are often observed concurrently with AIP. High levels of immunoglobulin G4 (IgG4) in the blood serum and affected tissues are typical of this diagnostic entity. We describe a case report of a 58-year-old female with findings of AIP (according to Asian criteria), IgG4-positive mastitis, and histologically verified Mikulicz syndrome. The effect of corticoid therapy supported the diagnosis of AIP and simultaneously led to the eradication of recurrent mastitis. To the best of our knowledge, this is the first reported case of concurrent findings of AIP and IgG4 mastitis. Our case report supports the concept of systemic IgG4 syndrome with multisystem involvement. Timely diagnosis and appropriate therapy can be effective in a high percentage of patients.

IgG4-related disease

The term “IgG4-related disease” encompasses several disorders described many years ago under various designations depending on the organ or system involved (e.g., Mikulicz syndrome, Riedel's thyroiditis, and retroperitoneal fibrosis). The clinical presentation varies widely, as one or more organs may be affected, usually in the same region of the body and either synchronously or metachronously. The main targets are the pancreas, bile ducts, salivary glands, lachrymal glands, mediastinal lymph nodes, and retroperitoneum. IgG4-related disease is rare, with an estimated incidence of 0.2 to 1/100 000 in Japan and no available incidence data in occidental countries. Men older than 50 years are predominantly affected. Serum IgG4 levels are often greater than 1.35g/L. Enlargement of the involved organs, which may be pseudotumoral, is due to a combination of infiltration by T cells and IgG4-expressing plasma cells, storiform fibrosis, and obliterative thrombophlebitis. Glucocorticoid therapy is effective but may be followed by relapses requiring the use of immunomodulating agents such as azathioprine, methotrexate and, more recently, rituximab. IgG4-related disease is not an autoimmune condition related to IgG4 autoantibodies, and neither does it involve immune complexes. Specific joint involvement has been reported in a very small number of patients.

History of Autoimmune Pancreatitis and Mikuliczs Disease

The concept of a new class of systemic disease, IgG4-related disease, was established from findings in patients with autoimmune pancreatitis and Mikuliczs disease, showing the involvement of other organs. Autoimmune pancreatitis presents as a swollen pancreas that mimics pancreatic cancer and was first recognized by its characteristic findings of lymphoplasmacytic sclerosing pancreatitis (LPSP) and irregular narrowing of the main pancreatic duct. It was later found to be associated with autoimmunity and with high serum IgG4 concentrations and IgG4-bearing plasma cell infiltration. Autoimmune pancreatitis is also characterized by the systemic distribution of extra-pancreatic lesions and shows similar pathological findings of IgG4-bearing plasma cell infiltration, suggesting that autoimmune pancreatitis is a systemic, IgG4-related disease. Mikuliczs disease is another type of IgG4-related disease, presenting as symmetrical, painless enlargement of the lacrimal and salivary glands. Mikuliczs disease was considered as a complex of symptoms, rather than a disease entity. Furthermore, this disease had been regarded as one manifestation of a more generalized symptom complex known as Sjogrens syndrome. Mikuliczs disease was later distinguished from Sjogrens syndrome by its high serum IgG4 concentration and several characteristic clinical features. Mikuliczs disease shows similar systemic organ involvements as autoimmune pancreatitis. At present, autoimmune pancreatitis and Mikuliczs disease are recognized as major constituents of IgG4-relatated disease. Keywords: Autoimmune pancreatitis, IgG4, IgG4-related disease, lymphoplasmacytic sclerosing pancreatitis, Mikulicz's disease, Sjogren's syndrome, Corticosteroid Treatment, Mikulicz's Syndrome

Mantle cell lymphoma presenting as Mikulicz syndrome

Mantle cell lymphoma presenting as Mikulicz syndrome

A Case of Mikulicz's Disease, IgG4-related Plasmacytic Syndrome, Successfully Treated by Corticosteroid and Mizoribine, Followed by Mizoribine Alone

We report a case of Mikulicz's disease, IgG4-related plasmacytic syndrome, successfully treated with corticosteroid and mizoribine, followed by mizoribine alone. A 56-year-old woman experienced persistent bilateral swelling in the eyelids and submandibular region accompanied by lymphadenopathy without pain. The patient was diagnosed as having Mikulicz's disease since her bilateral lacrimal glands showed persistent swelling and biopsy specimens of the submandibular glands and lymph node showed diffuse infiltration of lymphocytes and plasma cells. Symptoms and laboratory data were improved with corticosteroids (30 mg). However, after tapering prednisolone to 5 mg, submandibular swelling developed. Adding mizoribine resulted in clinical improvement. Prednisolone was tapered and finally discontinued. Since the levels of IgG4 in peripheral blood were increased even after corticosteroid therapy and IgG4 was positive in a submandibular gland specimen obtained before the start of corticoid therapy. Thus, this case may be associated with IgG4. The present case is the first case in which mizoribine was effective for Mikulicz's disease and IgG4-related plasmacytic syndrome.

Mantle Cell Lymphoma Presenting as Mikulicz Syndrome

A middle-aged woman presented with insidious onset swelling of bilateral lacrimal and parotid glands. Mikulicz syndrome is a symptom complex caused by a variety of systemic disorders like lymphoma, sarcoidosis, amyloidosis, human immunodeficiency virus infection, tuberculosis, etc. Biopsy from her lacrimal glands revealed mantle cell lymphoma. Although involvement of salivary and lacrimal glands as a part of generalized lymphomatous involvement is not uncommon, Mikulicz syndrome as a presenting manifestation of a lymphoma is very rare.

Mikulicz症候群を伴ったT cell CLLの1例

Mikulicz症候群を伴ったT cell CLLの1例

サルコイドーシスを基礎疾患としたミクリッツ症候群の１例

A 29-year-old woman with symmetrical bilateral swelling of the parotid, submandibular, and lacrimal glands--also called Mikulicz's symptoms--showed skin rash and pedal erythema. Serum IgG4 was within the normal range but serum ACE and lysozome were elevated. Incisional biopsy of the parotid glands and histopathological examination indicated sarcoidosis. This was complicated by uveitis and a low-grade fever. No facial nerve palsy was observed. Based on these findings, the definitive diagnosis was Mikulicz's syndrome associated with sarcoidosis. An alternative diagnosis was incomplete Heerfordt syndrome. The administration of steroid hormones, caused parotid, submandibular, and lacrimal glands swelling to disappear.

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