Kimura's Disease Research Articles

Assessing the Efficacy of Steroids as a Single Modality Treatment for Kimura Disease: A Retrospective Analysis.

Kimura disease is an idiopathic chronic inflammatory disorder, that usually affects the head and neck sites. The use of steroid for its management has been long reviewed in literature alongside immune suppression, but there are only few studies that compare the efficacy of steroid as a single modality treatment for the same. A middle-aged patient, hailing from southern state of India, presented to our outpatient clinic with right sided facial swelling for 2 years. Patient was diagnosed as a case of kimura disease of head and neck with cytological analysis and other investigations. Patient was managed medically with low dose oral corticosteroids and followed up for 6 months. This is a retrospective analysis of the efficacy of this single modality treatment. Patients with Kimura disease with no renal involvement, low dose oral corticosteroids can be tried as a single modality treatment, provided there are no contra indications for the same. Although long term follow up is essential to look for recurrence rates and associated long term benefits for the same.

Kimura's disease of periocular region: Report of two cases and review of literature

We report two patients with periocular Kimura's disease (KD). The clinical features and management of two histopathologically proven KD patients who underwent near-total mass excisions are discussed. Both were males (74 years and 55 years), having painless masses in the lacrimal sac region and upper eyelid, respectively. The masses were firm and adherent to surrounding tissues. Near-total mass excisions and histopathology showed dense germinal center hyperplasia with lympho-mononuclear infiltrates and skeletal muscle infiltration. Additional features of increased eosinophils and thin vessel vasculitis were suggestive of KD. Both were managed with oral steroids, and one recurrence with intralesional steroid injection, a novel treatment strategy for local recurrences in KD.

KIMURA’S DISEASE: Presenting as unilateral parotid mass

Objectives: Kimura’s disease (KD), or eosinophilic lymphogranuloma, is a rare chronic inflammatory condition with unclear etiology. We aim to present our experience with this condition and review the available literature. Methods: This report depicts a pathologically proven case of KD in a 44-year-old man with gradually increased swelling in his left parotid region for the past year, which was painless, and pruritus. Results: This report depicts a pathologically proven case of KD in a 44-year-old man with gradually increased swelling in his left parotid region for the past year, which was painless, and pruritus. Conclusion: This report depicts a pathologically proven case of KD in a 44-year-old man with gradually increased swelling in his left parotid region for the past year, which was painless, and pruritus.

Swelling of the salivary glands and sicca symptoms in Kimura's disease, arare rheumatological differential diagnosis with an indicatively high IgE serum level-Acurrent overview after literature search

Rheumatic and musculoskeletal diseases (RMD) include various diseases with sometimes rather different symptoms, some of which are locally confined and others show systemic features. Autoimmune phenomena, such as those occurring in Sjögren's syndrome, often cause symptoms such as xerostomia and xerophthalmia in association with inflammation of the salivary glands. The pathogenesis of these diseases is only partly clarified. This is similar to allergic diseases, which are otherwise clearly different with respect to the symptoms and pathomechanisms but swelling of the salivary glands can also rarely occur here. As this is apossible differential diagnosis of Sjögren's syndrome or also IgG4-associated diseases, and such acase was recently described, aliterature search was carried out in PubMed, the results of which are presented here and summarized in this article in abrief overview.

Kimura Disease Masquerading as Vascular Malformation - An Interesting Report.

Kimura disease is a chronic inflammatory disorder which is rare, cause is unknown and primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling in head and neck, blood and tissue eosinophilia and raised IgE levels. The etiology is thought to be manifestation of an aberrant allergic response. The main modalities of treatment are corticosteroids/ surgery or both. Here we report a case of 16 years old male who presented to our hospital with fluctuant swelling over the post auricular region of 06months duration. Imaging suggested vascular malformation, however the final diagnosis was found to be Kimura disease.

Angiolymphoid Hyperplasia with Eosinophilia of the External Auditory Canal.

Angiolymphoid hyperplasia with eosinophilia is a rare, benign, vaso-proliferative disease with a dense eosinophilic inflammatory infiltrate seen in the intradermal or subcutaneous region. It is also termed an epitheloid hemangioma, and it mostly affects females rather than males. It is seen as reddish to brown, fleshy, proliferative, papular, or nodular lesion affecting the head and neck region, most commonly the auricular and periauricular regions. The aetiology is unknown, but the most likely causes are trauma, infection, and hyperestrogenic condition like pregnancy or the use of oral contraceptive agents. It usually measures about 2-3cm in size. It should be differentiated from Kimura disease, which is characterized by chronic inflammation and large subcutaneous nodules in the head and neck region. Here is a rare case report of angiolymphoid hyperplasia in a middle-aged male patient involving the external auditory canal of the left ear.

A case of Kimura disease treated with dupilumab

A case of Kimura disease treated with dupilumab

Pathological significance of plasma cell infiltration in diagnosing lymph node diseases

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.

Kimura Disease: a Case Report in an Unlikely age group

Kimura disease is a rare chronic inflammatory disorder of unknown etiology. The disease is primarily seen in young Asian males and is characterized by painless subcutaneous swelling. It is often accompanied by renal involvement, predominantly manifesting as nephrotic syndrome. We describe a case of Kimura disease in a 69-year-old Indian male, presenting with left postauricular swelling. Clinical and histopathological characteristics are described along with a brief review of the literature

A common presentation of an uncommon pathology: Kimura disease.

Kimura's disease is a chronic, benign inflammatory condition of the subcutaneous tissue. It presents as painless, subcutaneous nodules of the head and neck, which are firm, painless, and may be single or multiple. It is most commonly seen in young adult Asian men. The nodes remain stable or may slowly enlarge over time. Elevated serum immunoglobulin E (IgE) levels, peripheral blood eosinophilia and lymphoid proliferation with eosinophilic infiltration on histopathological examination are the characteristic features. Spontaneous regression is usually seen. The diagnosis of Kimura's disease can be difficult and misleading, and it is important not to ignore histopathological features.

Facial Granular Lesions in a Patient With Kimura Disease Associated With Focal Segmental Glomerulosclerosis (FSGS).

Kimura disease is a benign subcutaneous chronic inflammatory disease of unknown etiology that is usually seen in young males. A 26-year-old Syrian adult, has suffered from focal segmental glomerulosclerosis for 10 years with no history of renal transplantation, complained of swellings in his preauricular area which was diagnosed as Kimura disease. There is no consensus regarding the optimal treatment for Kimura disease and the chosen treatment was surgery in the young patient with localized lesions. During 9 months of following up after surgical removing of the lesions, no recurrence was noticed.

CLINICAL CHARACTERISTICS AND OUTCOMES OF KIMURA DISEASE IN MALAYSIA: A CASE SERIES AND A LITERATURE REVIEW

Abstract: Kimura disease is a rare benign chronic inflammatory disorder of the soft tissue with predominant occurrences in young Asian male adults. However, there is limited information of Kimura disease in Southeast Asia. Hence, the clinical characteristics and outcomes of Kimura disease in Malaysia was investigated. Published Kimura disease cases from Malaysia were comprehensively searched in PubMed and Google Scholar up to December 2020 using the keywords “Kimura Disease” and “Malaysia”. Twenty-three papers were identified for review and case series of seven Kimura disease patients from a hospital in Malaysia were descriptively analyzed. A total of 60 cases were obtained from both sources. Eighty-seven percent were men with a male:female ratio of 6.5:1 and majority of the cases were Malays (77.1%). Median age of onset was 22.0 (IQR 12.5-31.5) years, while the median duration before diagnosis of Kimura disease was 2.0 (IQR 0.8-5.0) years. Head and neck region (95%) were the most frequently involved anatomical site. Peripheral eosinophilia was detected in 88.9% of the cases at presentation and renal involvement was observed in four (22.2%) patients. Surgery (57.5%) was the commonest first choice of treatment, followed by a combination of surgery and steroid (29.8%), steroid alone (10.6%), and a combination of steroid and immunosuppressive agent (2.1%). Local recurrences were observed in 28.6% of the cases. In conclusion, a much younger age of disease onset was found among Malaysians. A high recurrence rate of one in every four patients was observed, indicating the need for further evaluations of treatment strategies.

Multiomic landscape of immune pathogenesis in Kimura’s disease

Kimura's disease is a rare chronic inflammatory disorder characterized with subcutaneous masses, lymphadenopathy, and peripheral eosinophilia. So far, the disease pathogenesis remains hardly known. Here, we perform bulk-RNA sequencing and reveal a higher expression of transmembrane 176A (TMEM176A) with over-activated extracellular-signal-regulate kinase/mitogen-activated protein kinases (Erk/MAPK) signaling pathway in eosinophils of Kimura's disease compared with healthy controls. Flow cytometry analysis shows that the composition of lymphocytes, monocytes, and dendritic cell subsets are similar between Kimura's disease and healthy controls, which is further validated by scRNA-seq. Loss of S100 calcium binding protein P (S100P) is found in the CD24+ myeloid subset of Kimura's disease. Invitro functional assays show that S100P may participate in promoting reactive oxygen species (ROS) production in myeloid cells. Taken together, we are the first to study the immune pathogenesis systematically and demonstrate that Erk/MAPK signaling pathway might be a potential therapeutic target for Kimura's disease.

Kimura Disease in an Adult Indian Female, A Rare Presentation: A Novel Treatment with a Review of Literature.

Kimura disease (KD) is a chronic inflammatory disorder, which is quite rare and has an unclear and much debated etiology. KD is characterized by painless, nodular, subcutaneous swellings along with lymphadenopathy, encountered in the head and neck region more often. It is also known to affect major salivary glands. It is also associated with elevated Immunoglobulins (IgE) and peripheral blood and tissue eosinophilia. Kimura disease generally affects young Asian males. There is considerable difficulty in arriving at clinical diagnosis and often it is frustrating to both patients and the treating physicians because of the difficulty in its management. We are presenting and reporting a case of Kimura disease in a young adult Indian female patient who presented with a swelling in the left parotid region. We have successfully managed this patient medically with a unique combination of drugs, most of which have already been tried and reported in literature by various authors.

Kimura's Disease Diagnosed with Pleural Tumor.

Kimura's Disease Diagnosed with Pleural Tumor.

Kimura disease in children: A report of 11 cases and review of the literature.

Kimura disease (KD), also known as eosinophilic hyperplastic lymphoid granuloma, is a rare benign chronic inflammatory condition, which is featured with the painless progressive mass located in the subcutaneous area of the head and neck region, elevated peripheral blood eosinophils, and raised serum immunoglobulin E (IgE) levels. KD is uncommon in clinical practice, especially with low incidence in children, so it often leads to misdiagnosis or missed diagnosis in pediatric patients. The clinical data of 11 pediatric patients with KD in the authors' institution were retrospectively analyzed. There were 11 pediatric patients with KD enrolled in total, including 9 male patients and 2 female patients (sex ratio 4.5:1). The median age at diagnosis stood at 14 years (range 5-18 years), the initial symptoms in all patients included painless subcutaneous masses and focal swelling, the duration of symptoms ranged from 1 month to 10 years, and the average duration was 24.45 months. Six patients had single lesions, and 5 had multiple lesions. The highest proportion of lesion regions were parotid gland (n = 5, 31.3%) and retroauricular (n = 5, 31.3%), followed by cervical lymph nodes (n = 4, 25%), and others (n = 2,12.5; elbow n= 1; back n = 1). The eosinophil absolute count elevated in all patients, ranging from 0.71×109/L to 10.35 ×109/L (normal range 0.02-0.52×109/L). IgE levels were increased in all 7 patients who underwent serum immunoglobulin examination (normal range <100 IU/mL). Three patients received oral corticosteroid treatment while 2 relapsed. Three patients received surgical resection combined with oral corticosteroid treatment, and no patient relapsed. The other 3 patients received surgery and radiotherapy, surgery combined with corticosteroid and cyclosporin and corticosteroid combined with leflunomide respectively, and no patient relapsed. Base on the study, it is found that Kimura disease is rare and may have the atypical symptoms in pediatric patients, combination therapy is recommended to reduce recurrence, and long-term follow-up should be performed.

Kimura Disease: Rare Parotid Entity.

Kimura disease is a rare chronic inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling, blood and tissue eosinophilia and raised IgE levels. Early diagnosis of Kimura's disease may spare the patient from unnecessary invasive diagnostic procedure. We describe a case of Kimura disease in a 17-year-old male presenting with left submandibular swelling.

Refractory Kimura's disease accompanied with prurigo responding to dupilumab administration: A case report

Kimura's disease is refractory and sometimes has prurigo as a comorbidity. This report shows a case of Kimura's disease accompanied by prurigo and successfully treated both with dupilumab. Dupilumab might be considered for Kimura's disease with prurigo, which is resistant to conventional treatments. Kimura's disease is a disorder characterized by the formation of progressive masses in the soft tissues of the face, accompanied by an increase in peripheral eosinophil counts and serum total IgE levels. The etiology and pathogenesis of Kimura's disease are unknown. The condition is thought to involve a Th2-type immune response because it is frequently associated with allergic diseases, along with increased production of interleukin (IL)-4, IL-5, and IL-13 by peripheral blood mononuclear cells.1 Patients with Kimura's disease sometimes have prurigo as a comorbidity,2 and the symptoms of both are often refractory. Here, we report a case of Kimura's disease complicated by prurigo, in which dupilumab was effective for both. A 55-year-old female complained of post-auricular itchy swellings, and itchy nodules on her limbs and trunk, which had persisted for 14 and 10 years, respectively. She had a history of atopic dermatitis and bronchial asthma. The post-auricular swelling and body nodules were refractory to treatment with topical steroids and oral anti-histamines. Initial examination revealed dark purple, walnut-sized subcutaneous masses on the bilateral posterior auricles (Figure 1A), and multiple fingertip-sized elastic hard nodules on the limbs and trunk (Figure 1B). Blood tests revealed an increased eosinophil count (2.64 × 103/μL) and increased serum IgE levels (8861.0 IU/mL: <358), but normal serum TARC levels (235 pg/mL: <450). A skin biopsy from the post-auricular region showed lymphoid follicle formation and infiltration of the dermis by eosinophils and plasma cells (Figure 1C,D). A skin biopsy of an itchy nodule on the right forearm revealed findings consistent with prurigo. Based on the above, she was diagnosed with Kimura's disease complicated by prurigo. Three weeks of oral cyclosporine monotherapy (200 mg/day) failed to ameliorate Kimura's disease or prurigo. Subsequent combination therapies with prednisolone (PSL; 20 mg/day) and cyclosporine, and PSL plus PUVA-bath, also failed. Finally, as her skin phenotype fulfilled the diagnostic criteria for atopic dermatitis, she was given dupilumab (initial dose of 600 mg, followed by 300 mg every 2 weeks) concomitant with oral prednisolone. Both the post-auricular subcutaneous masses and the prurigo improved markedly, and the nodules almost flattened out after 11 months of administration (Figure 1E,F). Computed tomography also revealed an improvement in Kimura's disease (Figure 1G,H). The eosinophil count and serum IgE and TARC level decreased to 300/μL, 3354.6 IU/dL and 131 pg/mL, respectively. A year after starting dupilumab, the dose of PSL was reduced to 5 mg/day without recurrence of either disease. Treatment of Kimura's disease generally includes oral steroids, immunosuppressive agents, surgical resection, and local low-dose radiotherapy;2 however, adverse events can result in treatment discontinuation. In recent years, biologics such as dupilumab,3-5 omalizumab, an anti-IgE antibody,6 and mepolizumab, an anti-IL-5 antibody7-9 have been used successfully to treat Kimura's disease. As Kimura's disease is involved in Th2 immune response,1 blocking Th2 signaling with dupilumab may suppress its activity. In addition, dupilumab is effective against prurigo nodularis,10 which is also shown in our case. Thus, dupilumab should be a treatment option for Kimura's disease with prurigo that is resistant to conventional treatments. Yuta Koike received lecture fees from Sanofi. Hiroyuki Murota received lecture fees from Sanofi. Approval of the research protocol: N/A. This report is a single case report. Informed Consent: We obtained a written informed consent from the patient. Registry and the Registration No. of the study/trial: N/A. Animal Studies: N/A. Data sharing is not applicable to this article as no new data were created or analyzed in this study.

Kimura's disease

The analysis of published clinical observations on the diagnosis and treatment of patients with Kimura's disease, which is a rather rare chronic inflammatory disease of human soft tissues of unknown etiology, is presented. The onset of Kimura's illness is hardly noticeable. The disease proceeds sluggishly, with a varied clinical picture, most often with the formation of single or multiple subcutaneous nodules or tubercles and swelling in the head and neck on one or both sides. Other localizations of lesions are also described: limbs, orbit, eyelids, palate, pharynx, armpit, chest, inguinal region. The affected areas gradually increase in size over many months or years. Other clinical manifestations of Kimura's disease are presented in the form of edema of the extraocular muscle, juvenile temporal arteritis, sleep apnea in cases of damage to the larynx, and life-threatening coronary artery spasm in children. There are reports of other rare systemic clinical signs of Kimura's disease in the form of pruritus, eczema and rash. The disease is often associated with regional lymphadenopathy and/or salivary gland involvement, eosinophilia, and an increase in serum immunoglobulin E from 800 to 35,000 IU/mL. The disease may be accompanied by kidney damage in the form of glomerulonephritis and nephrotic syndrome. Most reported cases of Kimura's disease occur in young Asian men in their 20s and 30s, sporadically the disease is diagnosed in Europeans, North Americans, Caucasians, and Africans. Diagnosis of Kimura's disease is often clinically difficult. It is primarily based on excisional biopsy of the lesion or regional lymph nodes, and the histopathological findings are consistent regardless of the location of the lesion. Morphologically, Kimura's disease is characterized by the presence in biopsy specimens of such histological features as reactive follicular hyperplasia with prominent follicles surrounded by fibrous tissue. Interfollicular infiltrates are rich in plasma cells and eosinophils, which form diffuse eosinophilic microabscesses associated with granulomatous inflammation. The vessels are numerous, with an increase in postcapillary venules. At the same time, the blood vessels remain thin-walled, with cuboidal endothelial cells. Treatment issues are highlighted, and differences between Kimura's disease and angiolymphoid hyperplasia with eosinophilia are presented.

Common Postauricular Swelling Diagnosed as rare Kimura Disease.

Kimura disease is a rare benign chronic inflammatory condition of unknown aetiology that causes the subcutaneous tissue of head and neck to become swollen. It is more common in males, primarily seen in young Asian [1]. Here we are going to present a rare case report of 49 years old male diagnosed as kimura disease with a common presentation of postauricular swelling. It is characterised by peripheral eosinophilia FNAC suggestive of polymorphous population of lymphocytes, centrocytes, plasma cells, neutrophils and occasional eosinophils. Diagnosis confirmed by histopathological examination.