UNASSIGNED: Kimura\'s disease (KD) is a chronic, nonmalignant inflammatory disorder that primarily affects subcutaneous tissue. It is typically characterized by painless nodules in the head and neck regions, accompanied by elevated eosinophil and serum IgE levels. The purpose of this case study is to elucidate this rare disease, particularly in the Asian region and Syria, and to explore diagnostic and therapeutic methodologies with the objective of mitigating the number of undiagnosed patients suffering from this disease.
UNASSIGNED: A 23-year-old male patient presented to the Ear, Nose, and Throat (ENT) Department of the hospital with symptoms that had been initiated 7 months prior. The primary symptoms were pain and swelling in the left preauricular area, followed by subsequent swelling in the right preauricular area the next day. The patient experienced severe, intermittent pain, generalized pruritus, and systemic manifestations, including fever, chills, fatigue, malaise, anorexia, and a weight loss of 20 kg over the course of seven months. A fine-needle aspiration of the left parotid gland revealed the presence of lymphocytes at various maturation stages, with no evidence of abnormal cells. A diagnosis of KD was subsequently confirmed.
UNASSIGNED: To our knowledge, this case represents the second documented instance of KD in Syria. Furthermore, our case is among the extremely rare instances of KD in a patient without a history of renal transplantation.
UNASSIGNED: Further research is essential to ascertain the actual prevalence of this condition and to identify the most effective management strategies.

A male patient in his early 30s was diagnosed with bronchial asthma 3 years previously. He responded well to inhaled corticosteroids and long-acting beta-agonists. Approximately 18 months from the onset, the patient reported worsening symptoms. These symptoms included severe functional limitations, requiring frequent exposure to high-dose prednisolone. Mepolizumab was added to the treatment, leading to optimal control of bronchial asthma. Despite receiving seven doses of mepolizumab at monthly intervals, the patient developed cervical and postauricular lymphadenopathy and subcutaneous swelling of soft tissue. A cervical lymph node biopsy confirmed the diagnosis of Kimura disease. Following treatment with oral glucocorticoids and methotrexate, the patient experienced a complete resolution of symptoms. He has been in remission and off oral prednisolone for the last 13 months. In this case, we highlight the development of Kimura disease in a patient undergoing mepolizumab treatment.

UNASSIGNED: Kimura\'s disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%-18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD.
UNASSIGNED: A 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m2. Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin-angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels.
UNASSIGNED: Although uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury.

Kimura\'s disease (KD) is a chronic inflammatory disorder characterized by nontender lymphadenopathy involving the head and neck region. Renal involvement in KD is rare, especially in children. We report a 12-year-old boy who had been previously treated for classical KD and had presented with anasarca and oliguria after 4 years. There were no swellings or lymphadenopathy. The kidney biopsy revealed membranous nephropathy. Remission was achieved with oral prednisolone and tacrolimus therapy. This patient highlights the need to regularly monitor patients with KD for the evolution of renal diseases, even if lymphadenopathy regresses. Serial monitoring for eosinophilia, inflammatory markers, and urine examination is needed to help identify subclinical disease early and prompt initiation of specific therapy.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition characterized by nodular lesions predominantly in the head and neck region, often causing discomfort or pain. Treatment remains challenging because of its rarity and the lack of established guidelines. This report presents a case of ALHE affecting the earlobes that was successfully managed using ear lobule reduction surgery and subsequent intralesional steroid injections. A 31-year-old woman with a history of recurrent earlobe masses underwent a partial excision to avoid the loss of the earlobe. Histopathological examination confirmed Kimura disease, a variant of ALHE. Subsequent local methylprednisolone injections effectively controlled the remaining lesions, resulting in significant size reduction without notching. Various treatment modalities have been attempted for this condition; however, recurrence rates remain high. Surgical resection combined with intralesional corticosteroid injections is the preferred approach. In this case, a sub-antitragal groove technique for earlobe reduction was employed to preserve the lateral edge of the ear lobule, minimize the risk of deformity, and achieve a predictable outcome. The sub-antitragal groove technique offers an approach to reduce earlobe size without compromising aesthetics. Further research is required to elucidate the pathogenesis of ALHE and establish standardized treatment protocols for this rare condition.

暂无摘要。

Kimura disease (KD) is a rare, chronic, inflammatory condition, predominantly found in male patients of Asian ethnicity. It typically presents between 50-60 years of age and usually with bilateral disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) remains the main differential diagnosis, although histological analysis is essential in differentiating from other similarly presenting pathologies. In this case, we present an atypical case of unilateral orbital KD in a middle-aged, Caucasian, male gentleman and no evidence of regional lymphadenopathy along with a literature review of orbital KD and the differential diagnoses, histological features and management modalities available, adding to the sparse literature on the topic. At present, no recognised diagnostic criteria for KD are available, with histopathological analysis through incisional or excisional biopsy being the primary diagnostic method. Complete surgical excision with or without corticosteroid management remains the most common treatment modality although management is shifting to steroid-sparing immunomodulatory therapy. To the best of our knowledge, this is the first case to describe maintenance therapy of KD using mycophenolate mofetil.

UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

UNASSIGNED: To explore the clinical and imaging features of rare site Kimura\'s disease (KD).
UNASSIGNED: Retrospective analysis was conducted on the clinical manifestations, laboratory examinations, and imaging features of five patients with rare site KD. All imaging data, including the location, quantity, size, uniformity, boundary, and enhanced appearance of the lesion were evaluated by two independent radiologists.
UNASSIGNED: Of the five patients, four were asymptomatic, and one experienced localized skin itching. Four cases involved subcutaneous nodules in the upper arm, while one was in the inguinal region. The main manifestations were single (three cases) or multiple (two cases) subcutaneous nodules/masses, with three patients accompanied by local lymph node enlargement. Four patients exhibited elevated eosinophil counts in their peripheral blood. Four patients had lesions with vascular flow voids; in three of these, the lesions also showed prominent enhancement. Notably, the lesion in a 5-year-old did not show vascular flow voids but displayed significant enhancement. Additionally, two patients showed edema around the lesions.
UNASSIGNED: The presence of solitary or multiple subcutaneous nodules/masses in the upper arm or inguinal area, accompanied by lymph node enlargement, elevated eosinophils in the peripheral blood, and the observation of internal vascular within the lesion, can aid in the diagnosis of KD occurring in uncommon anatomical locations.

UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.