Kimura Disease of the Parotid Gland: Management Approach

Here we present a 43-years old Malay man with Kimura’s disease of the right parotid gland. Asian men have a predisposition for this chronic inflammatory disorder of unknown etiology mostly presenting at the head and neck region. The management in dealing with this patient is not straight forward as the presentation mimics a subcutaneous tumor-like mass in which main differential diagnosis includes lymphoproliferative disorders and parotid neoplasm. Moreover, cytological investigation is inadequate, and this necessitates the use intra-operative frozen section prior to tumor debulking. Without proper investigation, misdiagnosis can occur and potentially expose the patient to unnecessary extensive surgical procedures. Here we discuss step by step approach in managing this patient.

Kimura Disease: A Rare Cause of Head and Neck Tumour in Children- A Case Report

Background: Kimura disease is a rare inflammatory condition classically manifested as painless subcutaneous nodules in the head and neck region and associated with regional cervical lymphadenopathy and salivary gland involvement. Objective: The purpose of this report is to illustrate the diagnostic difficulty due to its rarity and non-specific clinical presentation. Case Presentation: We present a case of Kimura disease with bilateral eyelid swelling, parotid involvement, and cervical lymphadenopathy in a young boy. Computed Tomography (CT) showed ill-defined enhancing masses in both upper eyelids with lacrimal gland infiltration, multiple nodular lesions in both parotid glands, and bilateral enlargement of cervical nodes. After several multidisciplinary discussions and trials of medications, Kimura's disease was finally confirmed by histology examination. Conclusion: In conclusion, a rare disease such as Kimura must be considered earlier if the patient is not responding to the treatment.

Bilateral Posterior Auricular Masses: A Case of Kimura’s Disease

Kimura’s disease (KD) is currently considered a rare chronic inflammatory disease of unknown etiology. It is more common in the Asian population, especially in young and middle-aged men, and can involve the lymph nodes, salivary glands, and subcutaneous tissues. It has been reported in adults and children, and is often accompanied by elevated peripheral blood eosinophils and elevated serum immunoglobulin E (IgE). Herein, we report a case of KD in a 46-year-old man with bilateral masses behind the ears since childhood that had gradually enlarged over 40 years. The patient’s peripheral blood eosinophils were elevated, and interestingly, homocysteine levels were also elevated. After surgical resection of the bilateral posterior auricular masses, follow-up over 5 years indicated good recovery and no signs of recurrence.

Comparison between Kimura’s disease and angiolymphoid hyperplasia with eosinophilia: case reports and literature review

Kimura’s disease (KD) is a rare chronic inflammatory or allergic disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm. Their relationship has always been debated. This article reports two rare cases, one of each disease. One patient was a 48-year-old female that presented with a mass on her right mandible. She also had oedema erythema and wheals on her lower limbs. She was diagnosed with Kimura’s disease complicated with chronic urticaria. The second patient was a 23-year-old female that presented with multiple nodules of unequal size on the scalp. She was diagnosed with angiolymphoid hyperplasia with eosinophilia. The first patient recovered after being treated with surgical resection, glucocorticosteroids, cyclophosphamide and radiotherapy. The second patient underwent the first stage of surgical excision and is currently being followed-up. Comparison of the clinical and histopathological features of these two cases supports the theory that KD and ALHE are two separate disease entities.

Eosinophilic interstitial nephritis and cardiac insufficiency in Kimura’s disease: a case report

Background Kimura’s disease (KD) is a rare chronic inflammatory disease and the etiology remains uncharacterized. The typical manifestations are painless lymph node or subcutaneous masses. There is currently no report of prominent renal interstitial injury and cardiac insufficiency in KD. Case presentation A 45-year-old man was referred to our hospital with dark urine, subcutaneous masses in forehead and right retroauricular, multiple lymphadenopathy and unexplained cardiac insufficiency. Renal biopsy demonstrated eosinophilic interstitial nephritis. Laboratory tests revealed eosinophilia and a high level of serum IgE. A biopsy of cervical lymph node was performed and KD was diagnosed. Treatment with oral prednisone resulted in a decrease of eosinophil, serum IgE, improvement of cardiac function, and regression of the subcutaneous mass. Conclusions We describe an extremely rare KD case presenting with eosinophilic interstitial nephritis, cardiac insufficiency and significant response to prednisone. The clinicians should improve the disease awareness and find optimal treatment.

Rituximab Treatment in a Patient with Kimura Disease and Membranous Nephropathy: Case Report

Kimura disease (KD) is a chronic, inflammatory disorder with slowly developing subcutaneous tumor-like swellings, often occurring in the head and neck region. KD is diagnosed based on histology, elevated levels of immunoglobulin type E, and increased peripheral eosinophil granulocytes. KD may coexist with glomerular renal diseases, and this case report is based on a patient with KD-associated membranous nephropathy. Patients with membranous nephropathy without KD have demonstrated responsiveness to treatment with monoclonal anti-CD20 antibodies. This case report is the first to investigate the effect of rituximab treatment in a patient with KD-associated membranous nephropathy. A 30-year-old Italian man living in Denmark was diagnosed with Kimura’s disease based on subcutaneous nodules with eosinophil angiolymphoid hyperplasia. The patient was admitted to the hospital due to nephrotic syndrome. Serology showed eosinophil granulocytosis and negative PLA2-receptor antibody. Renal biopsy showed membranous nephropathy, and the patient was treated with systemic methylprednisolone followed by cyclosporin and then cyclophosphamide with only partial remission. Ultimately, treatment with intravenous rituximab was initiated, which resulted in overall remission and no nephrotic relapses at 30 months of follow-up. Thus, intravenous rituximab effectively decreased proteinuria and prevented nephrotic relapses in a patient with treatment-refractory membranous nephropathy due to KD.

Case Report: A Rare Case of Nasal Forehead Mass in Kimura's Disease

Background: Kimura's disease is a rheumatic immune disease and head and neck lymph nodes are often involved. A mass occurring in the nasal forehead is rare. Good prognosis after surgical resection by glucocorticoid therapy is more rare.Case Summary: We report the rare case of a nasal forehead mass in a 45-year-old male patient with Kimura's disease. The patient underwent resection of the mass in October 2018 in a local hospital and the postoperative pathology was unclear. He then underwent a second resection in our department in December 2019 mainly because growth of the mass was affecting his appearance. Postoperative pathology confirmed that the patient had Kimura's disease, and he accepted systemic treatment with prednisone. We followed the patient for 10 months after surgery. He is now recovering well and continues to be closely monitored during follow-up.Conclusion: It is rare that the painless mass in the nasal forehead is diagnosed as a Kimura's disease.After completely resection of the mass and systemic treatment with prednisone, the patient had a good outcome. We provide experience for the treatment of Kimura's disease in nasal forehead.