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.
Kimura Disease

ABSTRACT Kimura disease (KD) is a rare chronic inflammatory disorder of unknown aetiology that primarily affects the head and neck region with lymph node involvement. Young to middle-aged adult Asian males are predominantly affected. The most common presentation is painless subcutaneous swelling in the head and neck region, while proptosis or orbital involvement is very rarely reported. KD shares some features with other inflammatory and neoplastic disorders, including lymphoma; thus, investigations to confirm the diagnosis should not be delayed. Systemic corticosteroids are commonly used to treat KD and show an excellent response; however, the optimal treatment is still uncertain, and KD has a high recurrence rate. We describe the case of a patient with KD who presented with proptosis and post-auricular swelling, which responded well to oral prednisolone treatment.
Kimura Disease ; Exophthalmos

Imiquimod, a toll-like receptor agonist, is a topical immunomodulator that induces the production of several cytokines including interferon-alpha, which shows antifibrotic properties. We hypothesized that the antifibrotic effect of imiquimod would soften fibrotic skin lesions. Therefore, we applied topical imiquimod with topical tacrolimus or systemic acitretin in patients with refractory lesions of myxedema, angiolymphoid hyperplasia, and generalized discoid lupus erythematosus and confirmed improvement in fibrotic lesions in these patients. Thus, we conclude that use of imiquimod improves fibrotic skin lesions. This report describes our experience with the treatment of this condition along with a review of the related literature.
Acitretin ; Angiolymphoid Hyperplasia with Eosinophilia ; Cytokines ; Fibrosis ; Humans ; Hyperplasia ; Interferon-alpha ; Lupus Erythematosus, Discoid ; Myxedema ; Skin ; Tacrolimus ; Toll-Like Receptors

Hypereosinophilia, defined as an absolute eosinophil count of >1,500/μL, can be caused by a number of allergic, infectious, paraneoplastic and neoplastic disorders. In cases of hypereosinophilia with lymphoid proliferation, pathological confirmation is essential to exclude either myeloid or lymphoid malignancy. A 38-year-old woman with both cervical lymphadenopathies and peripheral blood eosinophilia visited our clinic. She had already performed core biopsy of lymph nodes and diagnosed as Kimura disease at a regional hospital. At the time of our clinic visit, there were no palpable cervical lymph nodes. The blood test showed hypereosinophilia with a high total IgE level. There was no evidence of tissue infiltration of eosinophils except for duodenitis with eosinophilic infiltration. Based on these findings, she was diagnosed as Kimura disease. She treated with high-dose systemic corticosteroid (1 mg/kg) and additional immunosuppressants sequentially used cyclophosphamide and cyclosporine. However, her eosinophilia waxed and waned, and a left inguinal mass was newly found. Excisional biopsy findings showed large atypical lymphoid cells with numerous eosinophilis, and immunohistochemistry showed CD3+, CD20−, CD30+ and anaplastic lymphoma kinase (ALK). The final diagnosis was ALK-negative anaplastic large cell lymphoma. We report a case of anaplastic large cell lymphoma with marked peripheral eosinophilia misdiagnosed as Kimura disease. In the case of hypereosinophilia with lymphadenopathy, it is necessary to differentiate hematologic diseases through immunochemical staining.
Adult ; Ambulatory Care ; Angiolymphoid Hyperplasia with Eosinophilia* ; Biopsy ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Duodenitis ; Eosinophilia* ; Eosinophils ; Female ; Hematologic Diseases ; Hematologic Tests ; Humans ; Immunoglobulin E ; Immunohistochemistry ; Immunosuppressive Agents ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic* ; Phosphotransferases

Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.
Adrenal Cortex Hormones ; Angiolymphoid Hyperplasia with Eosinophilia ; Arteritis ; Giant Cell Arteritis ; Humans ; Inflammation ; Recurrence ; Temporal Arteries ; Ultrasonography

Kimura disease (KD) is an immune-mediated chronic inflammatory disease of unknown etiology. KD has many complications associated with hypereosinophilia, including various forms of allergic reactions and eosinophilic lung disease. Additionally, hypereosinophilia is associated with hypercoagulability, which may lead to thromboembolic events. A 36-year-old man with KD presented with acute limb ischemia and coronary artery occlusion. He underwent thrombectomy, partial endarterectomy of both popliteal arteries, and coronary artery stent insertion. KD is a systemic disease that affects many organs and presents with thromboembolism and vasculitis. In a patient with KD, physicians should evaluate the vascular system, including the coronary arteries.
Adult ; Angiolymphoid Hyperplasia with Eosinophilia ; Coronary Artery Disease* ; Coronary Stenosis ; Coronary Vessels* ; Endarterectomy ; Eosinophils ; Extremities* ; Humans ; Hypersensitivity ; Ischemia* ; Lung Diseases ; Popliteal Artery ; Stents ; Thrombectomy ; Thromboembolism ; Thrombophilia ; Vasculitis

Kimura disease is a rare idiopathic chronic inflammatory disorder. It typically presents in the head and neck area, whereas bilateral involvement is unusual. Its diagnosis requires it to be differentiated from other inflammatory diseases and from head and neck tumors. Treatment methods include conservative management, steroid administration, radiotherapy, and surgery; however, no single treatment of choice has been established. Herein, we report an unusual presentation of Kimura disease with bilateral parotid involvement. This case was treated by surgical excision.
Angiolymphoid Hyperplasia with Eosinophilia* ; Diagnosis ; Head ; Head and Neck Neoplasms ; Neck ; Parotid Neoplasms ; Radiotherapy

Kimura disease is a rare idiopathic chronic inflammatory disorder. It typically presents in the head and neck area, whereas bilateral involvement is unusual. Its diagnosis requires it to be differentiated from other inflammatory diseases and from head and neck tumors. Treatment methods include conservative management, steroid administration, radiotherapy, and surgery; however, no single treatment of choice has been established. Herein, we report an unusual presentation of Kimura disease with bilateral parotid involvement. This case was treated by surgical excision.
Angiolymphoid Hyperplasia with Eosinophilia* ; Diagnosis ; Head ; Head and Neck Neoplasms ; Neck ; Parotid Neoplasms ; Radiotherapy

BACKGROUND: Kimura disease (KD) is a systemic soft-tissue disease that leads to formation of painless masses in lymph nodes, with the highest predilection for the head and neck and especially the parotid gland. KD lesions are characterized by marked eosinophil infiltration, production of IgE and increased expression of T-helper type 2 (Th2) cytokines (interleukin [IL]-4, IL-5, etc.). Skewing to a Th2 inflammation is also demonstrated in the peripheral blood, with elevated eosinophils and high IgE levels. It is thought that basophils may play important roles in orchestrating this Th2 inflammation via IL-4 production leading to the induction of IgE synthesis as well as eosinophil infiltration. However, there are no reports as yet on the role of basophils in KD. OBJECTIVE: The present study was performed to investigate the potential role of basophils in the pathogenesis of KD. In this context we also examined the expression of IL-4 in basophils in the KD lesions. METHODS: By immunohistochemistry using a monoclonal antibody against a basophil marker ProMBP1 we investigated the number and distribution of basophils in the KD lesions. By double immunohistochemistry we analyzed the colocalization of IL-4 in basophils. RESULTS: There was an increased number of basophils infiltrating the KD parotid gland lesions as compared to that in normal control parotid tissue. By double-immunofluorescence we found that approximately 7% of IL-4-positive cells in KD patients' parotid glands were basophils. CONCLUSION: Basophils may also play a role in the pathogenesis of KD, leading to the induction of IgE synthesis and eosinophil infiltration.
Angiolymphoid Hyperplasia with Eosinophilia ; Basophils ; Cytokines ; Eosinophils ; Head ; Immunoglobulin E ; Immunohistochemistry ; Inflammation ; Interleukin-4 ; Interleukin-5 ; Lymph Nodes ; Neck ; Parotid Gland

Kimura's disease is a rare, benign, slow-growing chronic inflammatory swelling with a predilection for the head and neck region and is almost always with peripheral blood eosinophilia and elevated serum IgE levels. It is endemic in Asian males and rare in Western people. Surgical excision of the lesion is the first line therapy. Drug and radiation therapy have to be considered for the refractory lesions.
Angiolymphoid Hyperplasia with Eosinophilia ; diagnosis ; Asian Continental Ancestry Group ; Eosinophilia ; pathology ; Humans ; Immunoglobulin E ; blood ; Inflammation ; pathology ; Male ; Neck ; pathology