Case Of Kimura Disease Research Articles

Kimura disease: A rare cause of cervical lymphadenopathy and a diagnostic challenge

Abstract: Kimura disease (KD) is a rare chronic inflammatory disorder characterized by multiple painless solitary head and neck lymphadenopathy often accompanied by peripheral eosinophilia and elevated serum IgE levels. Initially, the lesion was described in 1937 as a neoplasm (Kimm and Szeto) and first coined by Kimura, a Japanese scientist. Now it is well documented that it is a benign condition with unknown etiology. An allergic reaction and an alteration of the immune system remains one of the pathophysiological bases. Persistent antigenic stimulation following arthropod bites and parasitic or candida infection are also suspected. KD is a quite rare condition, affected Caucasians are very rare. It is found almost exclusively in Asian males in their 2nd to 4th decade of life (70–80%). There are only few cases of KD reported from Indian subcontinent. We present a case series of three patients of Kimura disease, presenting with painless cervical and parotid lymphadenopathy. All three patients underwent lymph node fine needle aspiration cytology (FNAC) showing no evidence of malignancy. This case series highlights the very rare yet important cause of cervical lymphadenopathy in young patients. Cervical lymphadenopathy can be misleading if not evaluated by histopathology and serum IgE levels.

Kimura Disease: A Detailed Analysis of Clinical and Radiological Manifestations in a Retrospective Case Series.

Kimura disease (KD) is a rare chronic inflammatory disease that affects mainly young Asian men and is characterized by painless subcutaneous masses, lymphadenopathy, and elevated serum IgE levels. Despite its benign nature, KD poses a diagnostic and therapeutic challenge due to its rarity and clinical variability. This study aimed to provide a comprehensive analysis of the clinical and radiological features of KD in a retrospective case series, to assess treatment outcomes, and to discuss the implications for diagnosis and management. We retrospectively analyzed four histologically confirmed cases of KD admitted to Zhejiang Provincial People's Hospital from January 2018 to October 2023. Clinical and radiological data were retrospectively analyzed, and imaging findings were analyzed by two neuroradiologists to determine lesion characteristics and contrast enhancement patterns. Our findings showed that the patients were predominantly male, with a mean age of 43 years and an age range of 13-71 years. All patients presented with painless subcutaneous masses and three of them had peripheral blood eosinophilia and elevated serum IgE levels. Radiographically, the lesions were predominantly ill-defined with heterogeneous enhancement, accompanied by subcutaneous fat atrophy. Complete surgical excision and oral corticosteroids were effective treatments, and no recurrence was noted during follow-up. KD should be considered in the differential diagnosis of painless subcutaneous masses in the head and neck region, especially in the presence of eosinophilia and elevated IgE levels. Our findings contribute to the understanding of KD's clinical and radiological spectrum and highlight the need for long-term follow-up due to the risk of recurrence.

The clinical and pathological features of Kimura disease in pediatric patients.

Kimura disease is characterized by inflammation, with its underlying causes remaining uncertain. There is a lack of comprehensive and systematic research on the pathology of this condition in pediatric patients. Our objective is to study the clinical and pathological attributes of Kimura disease in pediatric patients and investigate the potential diagnostic significance of immunoglobulin E (IgE) in this context. Clinical and laboratory information, pathological characteristics, and follow-up data were correlated to examine the distinctive features. Immunohistochemistry, acid-fast staining, and molecular assay were used to identify the presence of IgE and pathogens. We conducted an analysis of five cases of Kimura disease in pediatric patients at our hospital. The patients' ages ranged from 5 years and 7 months to 14 years and 2 months, with 4 (80%) being male. The most common site was the head and neck region, particularly the postauricular subcutaneous area. Eosinophilia was observed in four patients (80%), and two patients (40%) had elevated serum immunoglobulin E (IgE) levels. Histopathological changes included eosinophilic infiltrates, follicular hyperplasia, and the proliferation of postcapillary venules. Immunohistochemical results supported the reactive nature of the lymphoid process and IgE deposition in the follicle, while no specific pathogens were discovered by special staining. All patients underwent surgical excision, and none experienced recurrence in their original location. Children with Kimura disease show distinct eosinophilic and IgE alterations in both laboratory findings and pathological features. The application of immunohistochemical staining of IgE could serve as a promising marker for diagnosing Kimura disease.

Concurrent Warthin tumor and Kimura disease: a case report

BackgroundWarthin tumor (WT) is a common benign salivary tumor of the parotid gland. Clinically, it occurs in men in their fifth to seventh decades who typically smoke cigarettes. WTs have been reported with different head and neck neoplasms and other salivary gland tumors within the same or another salivary gland. Kimura disease (KD) is a rare chronic inflammatory disease with unknown etiology affecting young to middle-aged Asian men. KD presents as an asymptomatic nodule in the head and neck area, with regional lymphadenopathy and salivary gland involvement.Case presentationA 64-year-old Arabic man presented with a 10-year history of an asymptomatic swelling of the left face. Computed tomography showed a well-defined, multicystic mass with heterogeneous enhancement. The resected mass was composed of two distinct components. There was a well-demarcated proliferation of papillary and cystic oncocytic epithelium with lymphoid stroma, consistent with WT. Some areas exhibited sclerotic fibrosis, with multiple lymphoid follicles showing folliculolysis, follicular hyperplasia, and eosinophilic infiltrate. The patient’s immunoglobulin E level serum was elevated, confirming a coexisting KD. The patient underwent a left superficial parotidectomy, with no recurrence at a 30-month follow-up.ConclusionThis report describes the first concurrent case of WT and KD in the parotid gland.

Kimura Disease: A Diagnostic Perplexity

Introduction: Kimura disease (KD) is a rare condition of unknown etiology and pathogenesis. This is more common in young males of Asian descent. The most common clinical presentation is presence of unilateral subcutaneous mass in the head and neck region, especially in post-auricular area. We present a case of KD in 26- year- old Asian man who presented with subcutaneous mass behind both auricles. Case report: A 26- year- old man presented to ORL-HNS outpatient department of tertiary care centre with chief complaint of mass behind both ear for 5 months. Incisional biopsy was performed under local anesthesia. Histopathological examination report confirmed the case as KD. Patient was managed with intralesional triamcinolone injection. Conclusion: Kimura disease, though rare, should be considered in differential diagnosis of head and neck subcutaneous masses. A high-index of suspicion and tissue biopsy confirmation is essential to diagnose this condition.

Kimura's disease: A clinicopathological study of 23 cases.

Kimura's disease (KD) is an uncommon lymphoproliferative fibroinflammatory disorder. Patients present with head and neck subcutaneous nodules with or without lymphadenopathy. Peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels are typical. This study was designed to delineate the clinicopathological features, pattern of care, and disease course of 23 Taiwanese patients with KD. We retrospectively analyzed the clinical data of 23 consecutive cases (16 male and 7 female; age at diagnosis: 12-77 years) of KD diagnosed at our institution from 2015 to 2020. The median time from presentation to diagnosis was 1 month. Twenty-one patients presented with unilateral or bilateral head and neck masses. The remaining two presented with right flank and right arm lesions, respectively. Peripheral blood eosinophilia was observed in nine, and elevated IgE levels were observed in four. All were diagnosed using either excisional or core-needle biopsy. Seven patients underwent fine needle aspiration without a diagnostic yield. Salivary gland and lymph node involvement was observed in three and seven patients, respectively. Most lesions showed tissue eosinophilia (100%) and florid follicular hyperplasia (78.26%). Three cases had histological KD-IgG4-RD overlap and three had comorbid IgG4-RD were recognized. Thirteen patients underwent surgical resection, one received adjuvant therapy, and two received prednisolone monotherapy. KD should be considered in patients with subcutaneous masses, eosinophilia, and elevated IgE levels. Biopsy remains the gold standard of diagnosis. Increased recruitment of IgG4+ plasma cells is a common feature. Consideration of IgG4-RD in all KD patients may be prudent.

Cervico-facial Localization of Kimura’s Disease: Two Case Reports and Literature Review

Kimura’s disease is a very rare chronic inflammatory pathology of unknown etiology. It is actually an allergic or autoimmune reaction process involving blood vessels, lymphocytes and eosinophils. We report a first case of Kimura disease with cervical localization in a 37-year-old male Patient who saw the appearance of a swelling of the right parotid region evolving for 2 years. The patient benefited from a total right parotidectomy, the anatomopathological study of the tumoral piece came back in favor of Kimura’s disease. A second case of 20-year-old male Patient who saw the appearance of a 3 swelling of the right pre-auricular region, sub mental region and left cheek region evolving for 5 years. The patient underwent a biopsy of submental swelling, the anatomopathological study confirmed the diagnosis of Kimura’s disease. The patient benefited from a Corticosteroid therapy. The evolution was spectacular, with no recurrence at 12 months follow-up. Kimura’s disease is characterized clinically by subcutaneous nodules of cervicofacial localization, increased volume of salivary glands and satellite adenopathies. Its diagnosis is histological based on the identification of follicular hyperplasia with eosinophilic polynuclear abscesses. Treatment is surgical most often but in some cases Corticosteroid therapy is proposed.

A comparative study of Kimura's disease and IgG4-related disease: similarities, differences and overlapping features.

To compare the fibroinflammatory diseases Kimura's disease (KD) and immunoglobulin (Ig)G4-related disease (IgG4RD) and to explore their possible relationship. Forty-six cases of KD and 29 IgG4RD from our institution diagnosed from 2011 to 2020 were studied. They were compared with each other on clinical, pathological and immunohistological features. There were similar clinical features, except that IgG4RD affected an older patient population, with more frequent salivary gland involvement and KD affected head and neck lymph nodes, and showed blood eosinophilia more frequently than IgG4RD. IgG4RD exhibited frequent storiform fibrosis and obliterative phlebitis, while KD showed more frequent tissue eosinophilia, eosinophilic abscess, germinal centre eosinophilic deposit and vascularisation. Twenty to 30% of KD had more than 50 IgG4+ plasma cells (PC) per high-power field (HPF) and IgG4/IgG+ PC ratio exceeding 40%. These parameters, however, occurred in 100% of IgG4RD. Significantly more KD had >10 IgE+ PC/HPF and lymphoid germinal centre IgE reticular staining compared to IgG4RD. All these histological and immunohistological features are overlapping in the two diseases, although they differed with statistical significance. Our study confirmed that there is significant overlap in clinical, pathological and immunohistological features between KD and IgG4RD. It is important to recognise these overlapping features, and correlation with a clinicopathological picture is required in differential diagnoses. The overlapping features also suggest a possible close relationship between KD and IgG4RD, which could represent different facets of a continuous fibroinflammatory disease spectrum.

Kimura's Disease: A Confounding Condition.

Kimura disease (KD) is a chronic inflammatory lymphoproliferative disorder of unknown etiology and unsure pathogenesis. It primarily involves the head and neck region, presenting as an asymptomatic unilateral soft-tissue mass in the head and neck, frequently involving subcutaneous tissues, major salivary gland, lymph nodes. Peripheral blood eosinophilia, elevated serum immunoglobulin E (IgE) levels, and the microscopically lymphoid proliferation with eosinophilic infiltration are the characteristic features. We report a case of KD in a 22-year-old Indian male who presented with an asymptomatic parotid gland enlargement with lymphadenopathy. The clinical presentation was suggestive of the benign lesion but histopathology, as well as microscopic findings, allowed us to make a definitive diagnosis of KD. The patient was treated with steroids and antihistamine and showed no recurrence on follow-up.

Kimura Disease of Parotid Gland

Kimura disease is a rare, benign, chronic inflammatory disorder. Characteristic features of the disease include painless subcutaneous mass in the head and neck region, elevated serum eosinophilia and markedly raised serum immunoglobulin E. Herein, we report a case of Kimura disease in a patient who presented with a parotid mass and received both medical and surgical management. A course of high dose oral prednisolone and subsequently parotidectomy were the important elements of the management.International Journal of Human and Health Sciences Vol. 04 No. 01 January’20 Page : 63-66

The clinicopathological characteristics of Kimura disease in Chinese patients.

Kimura disease (KD) is a rare idiopathic inflammatory disorder of unknown etiology. Unusual presentations of KD might cause diagnostic difficulty or be misdiagnosed as malignancy if clinical suspicion is insufficiently high. Here, we aimed to determine the clinicopathological features of Chinese KD patients to reveal further insights into the natural history and treatment of this disease. The clinical data of 46 cases of KD diagnosed at Peking Union Medical College Hospital from January 1980 to December 2018 were analyzed retrospectively through case record review. Of 46 cases, 40 were male and six were female. The age at onset ranged from 2 to 56years (median 27years). All patients presented with either single (26.1%) or multi-focal (73.9%) subcutaneous masses. Twenty-nine (63.0%) cases presented with head and neck subcutaneous masses, and 9 cases (19.6%) involved different parts of the body. Parotid, submandibular, and lacrimal gland involvement occurred in 17 (37.0%), 3 (6.5%), and 2 cases (4.3%), respectively. Nephrotic syndrome was present in three cases (6.5%), and thromboembolism was present in five cases (10.9%). During follow-up, thirteen patients (13/28, 46.4%) relapsed over 1-13years (median 8.5years). The recurrence rate in patients receiving corticosteroids, surgery, and combined surgery and radiotherapy was 30.8%, 66.7%, and 50.0%, respectively. One patient was diagnosed with T cell lymphoma 1year after diagnosis of KD. KD is characterized by subcutaneous masses but it is also a systemic disease. Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.Key Points• Kimura disease (KD) is a rare inflammatory disorder of unknown etiology that is endemic in Asia.• Clinicians must regard and manage KD as a systemic disease.• There is no consensus on optimal treatments and further studies are necessary to improve outcomes.• Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.

Concurrent Kidney Glomerular and Interstitial Lesions Associated with Kimura’s Disease

Background: Kimura disease (KD) is a chronic benign inflammatory disorder that is usually manifested as a deep, subcutaneous mass with or without regional lymphadenopathy in the head and neck region. Various types of glomerulonephritis are associated with KD, including minimal change disease (MCD), membranous glomerulopathy (MN), and immunoglobulin (Ig)A-nephropathy. Kidney interstitial lesion associated with KD is rarely reported. The aim of this study was to expand the spectrum of kidney lesions associated with KD. Methods: From 2007 to 2016, 12 cases of KD with kidney lesions were retrospectively reviewed. Pathological examinations included hematoxylin and eosin staining, periodic acid-schiff staining, periodic acid-methenamine silver staining, and Masson staining, immunofluorescence, and electron microscope analyses. Results: Anatomic sites of subcutaneous involvement included head and neck area, arm, and groin. Most cases had elevated IgE level and peripheral eosinophilia. Nephrotic syndrome was the most common kidney manifestation. Pathological results showed 3 types of glomerulonephritis, including 9 cases of MCD, 2 cases of IgA nephropathy, and 1 case of MN. Of note, 4 MCD cases showed concurrent glomerular and interstitial lesions associated with KD, including 2 distinct patterns: (1) a diffusely eosinophilic and lymphatic infiltration similar to KD lesion developed elsewhere and (2) exclusively eosinophilic infiltration in the interstitium. Eight out of 10 patients were responsive to steroid treatment and had complete remission of proteinuria and recovery of kidney function. Conclusion: Our report suggested that, in addition to glomerulonephritis, interstitial lesions may also be associated with KD. Pathologist should pay special attention to differentiated diagnosis when such pathological changes are identified in patients with subcutaneous mass or cervical lymphadenopathy with concurrent kidney manifestation.

A Case of Kimura Disease with a Bulky Preauricular Mass

A Case of Kimura Disease with a Bulky Preauricular Mass

Minimal change disease and Kimura's disease responding to tacrolimus therapy

Kimura's disease (KD) usually presents with a subcutaneous swelling and associated lymphadenopathy in the periauricular area. KD has a tendency to involve the kidneys. Proteinuria is reported in 12%-16% of cases, and around 60%-70% of them develop nephrotic range proteinuria. We are reporting a case of KD which developed around 12 years later in a patient of biopsy-proven steroid responsive minimal change disease. Recurrent swellings of KD and subnephrotic range proteinuria responded to low-dose tacrolimus therapy (0.05 mg/kg).

Kimura's disease associated with IgA nephropathy: A case report.

Kimura's disease (KD) is a rare chronic inflammatory disorder of unknown etiology that manifests as painless subcutaneous lesions in the head and neck. It primarily affects young Asian males and is characterized by peripheral eosinophilia and elevated levels of serum Immunoglobulin (Ig)E. Histologically, a variety of renal lesions have been observed in patients with KD, although IgA nephropathy is rarely reported. In the current study, a case of KD with an atypical manifestation accompanied with IgA nephropathy was reported in a middle-aged Chinese man. The subcutaneous lesion was located on the elbow of the patient. Although the recurrence rate of KD-associated nephrotic syndrome is high, the patient has not experienced relapse following 6 months of pulse treatment with methylprednisone and cyclophosphamide. In conclusion, methylprednisone combined with cyclophosphamide is effective in the treatment of KD-associated IgA nephropathy, and long-term administration of methylprednisone may achieve the effect of preventing KD recurrence.

An MRI Investigation of Kimura Disease (KD) in Upper Extremity Soft Tissues: Two Case Reports

: Kimura disease (KD) is a rare chronic granulomatous inflammation disease with unknown causes, which is common in young Asian males. Local lymph node drainages are characteristic of the disease, the most common of which occur in the salivary glands. Other reported drainages occur in the axilla, popliteal fossa, groin, and upper extremities. Unfortunately, there not many characteristic manifestations that typically present in patients with KD. Sometimes only increased peripheral blood eosinophils and serum IgE concentration are the only positive presentations. Therefore, it is very difficult for physicians to diagnose KD. We report herein on two cases of KD. Both of them presented in the soft tissues of the upper extremities. Special attention was given to the MRI findings of the two patients with a view to disclosing specific characteristics that should help in the realization of clinical diagnosis.

Kimura's disease

Kimura's disease (KD) is a benign chronic inflammatory disorder attributed to an immune-mediated hypersensitivity which often presents as a tumor-like swelling in the head and neck region with or without lymphadenopathy, associated with hypereosinophilia and elevated serum immunoglobulin E. Most cases have been described predominantly in Chinese and Japanese people. KD has been confused with angiolymphoid hyperplasia with eosinophilia, from which it probably should be distinguished as a separate entity. The course is usually benign except for the potential cosmetic disfigurement. The diagnosis may be suggested by a fine-needle aspirate but is established by a biopsy. A case of KD in a 42-year-old male with recurrent left submandibular swelling is presented together with a brief review of the literature.

Expression of the interleukin-21 and phosphorylated extracellular signal regulated kinase 1/2 in Kimura disease

ObjectiveTo investigate the expressions of interleukin (IL)-21 and phosphorylated extracellular signal regulated kinase 1/2 (pERK1/2) in Kimura disease (KD) and to correlate the findings with clinical and prognostic variables.MethodsImmunohistochemical analysis...

Kimura's disease of the right cheek: A case report.

Kimura's disease (KD), a chronic inflammatory disease of uncertain etiology, manifests as a painless subcutaneous swelling in the head and neck region that involves major salivary glands and regional lymph nodes. To date, the majority of cases of KD have been documented in Asian males aged 20-30 years. However, the number of reported cases of KD involving the oral and maxillofacial area is limited, and since the masses appear similar to cysts or benign tumors, the establishment of an accurate pre-operative diagnosis is challenging. The accurate diagnosis of KD is considered to require surgical excision followed by histopathological examination. In the current case, a 39-year-old man was admitted to hospital in October 2011 with a swelling evident on his right cheek. Surgical excision was performed, and histopathological observation was carried out. The formation of a lymphoid nodule accompanied by the vigorous proliferation of small blood vessels, eosinophilic infiltration and thickened cell walls were observed. No sign of recurrence of the mass has yet been observed, on the basis of the telephone follow-up interviews. These findings provide a novel insight useful in the diagnosis of KD in the oral and maxillofacial area.

Retrospective Multicentric Study of 25 Kimura Disease Patients: Emphasis on Therapeutics and Shared Features with Cutaneous IgG4-Related Disease

Background: Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce. Objectives: Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD). Methods: Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD. Results: Patients were mostly male (84%), median-aged 42 years with lymph node, lacrimal/salivary gland and kidney involvements in 45, 24 and 12%, respectively. Surgical excision had 100% complete response and 60% relapse. Oral corticosteroids had 100% response with 50% relapse. Thalidomide, cyclosporine or interferon-α had 100% response, but 100, 20 and 50% relapse, respectively. KD showed clinicopathological similarities with 27 published cases of cutaneous IgG4-RD. Conclusion: Surgery may be used in resectable KD cases, whereas cyclosporine or thalidomide may represent interesting alternatives to oral corticosteroids in other cases. KD shares features with cutaneous IgG4-RD.