Increased blood group 2 innate lymphoid cells are involved in blood eosinophilia and itching in Kimura disease

Background: Kimura disease (KD) is a rare, chronic inflammatory disorder characterized by blood eosinophilia, general itching, and subcutaneous head and neck mass lesions; however, the etiology of this disease is unknown. We hypothesized that group 2 innate lymphoid cells (ILC2s) in peripheral blood may play an essential role in the pathogenesis of KD. Methods: The prevalence of blood ILC2s and their ability to produce interleukin (IL) -4, IL-5, IL-13, and IL-31 in patients with KD were compared with those in control subjects and in patients with house dust mite (HDM) -induced allergic rhinitis (AR). Changes in blood ILC2 prevalence, blood eosinophilia, and clinical symptoms after surgery and steroid therapy were evaluated. Results: Blood ILC2 prevalence in patients with KD were eight times and six times higher than those in control subjects and in patients with AR, respectively. There was a strong positive correlation between ILC2 prevalence and blood eosinophilia. Patients with KD showed increased serum IL-13 and decreased IL-31 levels. KD patient-derived blood ILC2s produced large amounts of IL-5 and IL-13 in response to prostaglandin (PG) D and leukotriene (LT) D , compared to ILC2s derived from control subjects and patients with AR. Surgery and systemic steroid therapy ameliorated general itching with a concomitant decrease in blood ILC2s and blood eosinophilia. Upon disease recurrence, blood ILC2 prevalence and blood eosinophilia increased concurrently with general itching. Conclusion: Increased blood ILC2s may be involved in blood eosinophilia and general itching through the production of IL-5 and IL-13 in patients with KD.

Kimura Disease as a Rare Cause of Proptosis: A Case Report

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: 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.

Kimura Disease With Allergic Bronchopulmonary Aspergillosis: A Case Report

Background: Kimura disease (KD) is a rare chronic idiopathic condition of unknown etiology that is prevalent in Asian males. It often causes subcutaneous lumps and enlarged lymph nodes, especially in head and neck region. But KD is also a systemic disease that can involve multiple organs, such as the kidneys and skin. Case presentation: Here, we report a 62-year-old Chinese man who was characterized by paroxysmal cough, enlarged inguinal lymph nodes, recurrent skin itching, and elevated IgE antibodies specific to A. fumigatus. After a comprehensive inspection, the final diagnosis for this patient was KD with Atopic Bronchopulmonary Aspergillosis (ABPA). Conclusions: The age of onset and the location of the lump involved were not the most common. This study described the patient’s diagnosis and treatment process. The coexistence of the two special diseases will provide clinicians with some enlightenment on the etiology of KD. Moreover, we hope to arouse the attention of multidisciplinary team to explore the potential relationship between KD and ABPA. It will contribute to preventing the misdiagnosis and missed diagnosis of KD.

Clinical, Pathological, Laboratory Characteristics, and Treatment Regimens of Kimura Disease and Their Relationships With Tumor Size and Recurrence

Objective: As of date, Kimura disease (KD) has an unclear etiology, no accepted diagnostic standard, and no definite treatment regimen. In this study, clinical and pathological laboratory characteristics and treatment regimens of patients with KD with different tumor sizes and status of tumor recurrence were analyzed. This was performed to identify the factors, which determine tumor size and recurrence, and to identify effective treatment methods for patients with KD.Methods: A total of 33 hospitalized patients with a definite diagnosis of KD were enrolled in this study.Results: There were 15 patients (45.5%) with a maximum tumor diameter of <3 cm. There were no statistically significant differences in age, gender, clinical symptoms, lesion sites, laboratory indicators, and treatment regimens among patients with a maximum tumor diameter <3 cm or ≥3 cm (P > 0.05). Among the 25 patients who completed the follow-up, there were 18 patients (72%) who had a recurrence of KD. There were no statistically significant differences in age, gender, clinical symptoms, the maximum tumor diameter, lesion sites, laboratory indicators, and initial treatment regimens between patients with or without the recurrence of KD (P > 0.05). There was a statistically significant difference in systolic blood pressure (SBP) between patients with or without the recurrence of KD (P < 0.05). All patients who received only surgical treatment had disease recurrence, 33.3% of patients who received prednisone therapy had no disease recurrence, and 37.5% of patients who received combination therapy showed recurrence.Conclusion: The current study summarized clinical manifestations, pathological features, laboratory indicators, and treatment regimens of patients with KD. There were no significant differences in these aspects among patients with different tumor sizes, and there was no significant difference in these aspects except in the SBP between patients with or without the recurrence of KD, indicating that SBP is a significant clinical factor affecting disease recurrence in patients. Combination therapy with prednisone was found to be superior to surgical treatment.

Kimura Disease of the Nose

Introduction: Kimura disease is a rare chronic inflammatory disorder affecting the subcutaneous tissue. It is of unknown etiology mainly occurring in head and neck region. Case report: A 34-year-old Malay lady presented with a gradually enlarging, painless right nasal bridge mass for one year duration. Clinical examination revealed a firm, immobile swelling measuring 2 cm x 2 cm. Nasal endoscopy showed no extension of the mass into the right nasal cavity. Surgical excision was performed where histopathological examination reported as finding consistent with Kimura disease. Conclusion: Kimura disease is extremely a rare benign disease, which can affect the head and neck structures. Manifestation at the nasal region is an extremely rare occurrence. Histopathological examination is needed to diagnose the disease.

Case Report: Hyper IgE, but Not the Usual Suspects–Kimura Disease in an Adolescent Female

Elevated immunoglobulin E (IgE) levels can be associated with infectious, allergic and inflammatory disorders, and rarely as a manifestation of an inborn error of immunity. Here we report the case of an adolescent female who presented with a gradually enlarging neck mass, lymphadenopathy, eosinophilia and highly elevated IgE levels. Laboratory and histopathologic evaluation revealed an unlikely diagnosis of Kimura Disease. We discuss the differential diagnosis of a neck mass with prominent eosinophils on histology, and review support for T-helper type 2 (Th2) cell activation and hyper-IgE in Kimura Disease.