kimura disease

木村病
  • 文章类型: Case Reports
    血管淋巴样增生伴嗜酸性粒细胞增多(ALHE)是一种罕见的,良性,血管增生性肿瘤。我们报告了一名25岁的女性患者,她于2021年在Rustaq的皮肤科诊所报告,阿曼,有多个,分组,左侧颞枕区持续6个月的红斑圆顶状丘疹和结节。活检结果与ALHE的诊断一致,有皮脂腺导管中蠕形螨感染的证据。在多次冷冻治疗和局部应用甲硝唑凝胶治疗7周后,患者表现出明显的改善。这种情况表明,头皮营养不良症可能是ALHE发展的新触发因素。
    Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, vasoproliferative tumour. We report a 25-year-old female patient who reported in 2021 to a dermatology clinic in Rustaq, Oman, with multiple, grouped, erythematous dome-shaped papules and nodules of 6 months duration on the left temporo-occipital region. Biopsy findings were consistent with a diagnosis of ALHE with evidence of Demodex mite infestation in the sebaceous ducts. The patient demonstrated significant improvement following 7 weeks of treatment with multiple cryotherapy sessions and topical application of metronidazole gel. This case suggests that scalp demodicosis may represent a novel trigger for the development of ALHE.
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  • 文章类型: Case Reports
    Kimura病(KD)是一种罕见的良性慢性炎症,主要影响亚洲男性。它的特征是头颈部的皮下组织肿块,淋巴结肿大,血嗜酸性粒细胞增多,血清总IgE水平升高。在这份报告中,我们描述了一个年轻的越南女性罕见的KD病例。一名31岁的越南妇女被送往医院,双侧脸颊有2个肿块,左耳后有1个肿块,持续了15年,复发性皮肤瘙痒,血清总IgE水平升高,血嗜酸性粒细胞增多.没有记录个人或家庭的病史。我们对耳后肿块进行了切除活检,发现滤泡增生伴小血管增生,淋巴滤泡中嗜酸性粒细胞弥漫性浸润,和几个嗜酸性微脓肿。经过全面审查,该患者的最终诊断为KD和特应性皮炎合并症.总之,KD不仅限于男性,正如这份报告所证明的。组织病理学检查在KD的诊断中起着重要作用。该病例说明了KD的特征性描述,并强调了亚洲女性对这种罕见疾病的认识。
    Kimura disease (KD) is a rare benign chronic inflammatory condition that predominantly affects Asian males. It is characterized by subcutaneous tissue masses in the head and neck region, enlarged lymph nodes, increased blood eosinophilia, and elevated serum total IgE levels. In this report, we describe a rare case of KD in a young Vietnamese female. A 31-year-old Vietnamese woman presented to the hospital with 2 masses in the bilateral cheeks and 1 mass behind the left ear that persisted for 15 years, recurrent skin itching, elevated serum total IgE levels, and increased blood eosinophilia. No medical history of the individual or family was recorded. We performed an excision biopsy of the postauricular mass that revealed follicular hyperplasia with small vessel hyperplasia, diffuse infiltration of eosinophils in lymphoid follicles, and several eosinophilic microabscesses. After a comprehensive review, the final diagnosis for this patient was KD and atopic dermatitis comorbidity. In conclusion, KD is not limited to males, as this report demonstrated. The histopathological examination plays an important role in the diagnosis of KD. This case illustrated the characteristic description of KD and highlights the need for awareness of this rare disease in Asian women.
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  • 文章类型: Case Reports
    木村病(KD)是一种慢性疾病,主要影响皮下组织的非恶性炎症性疾病。它的典型特征是头颈部无痛结节,伴有嗜酸性粒细胞和血清IgE水平升高。这个案例研究的目的是阐明这种罕见的疾病,特别是在亚洲地区和叙利亚,并探索诊断和治疗方法,目的是减少患有这种疾病的未确诊患者的数量。
    一位23岁的男性患者出现在耳前,鼻子,以及7个月前开始出现症状的医院喉部(ENT)。主要症状是左耳前区疼痛和肿胀,随后第二天右耳前区域肿胀。病人经历了严重的,间歇性疼痛,全身性瘙痒,和系统性表现,包括发烧,发冷,疲劳,萎靡不振,厌食症,在七个月的过程中体重减轻了20公斤。左腮腺的细针抽吸显示在各个成熟阶段存在淋巴细胞,没有异常细胞的证据.随后证实了KD的诊断。
    据我们所知,此案代表了叙利亚境内第二个记录在案的KD实例。此外,我们的病例是在没有肾移植史的患者中极为罕见的KD病例之一.
    进一步的研究对于确定这种情况的实际患病率和确定最有效的管理策略至关重要。
    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.
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  • 文章类型: Case Reports
    3年前,一名30多岁的男性患者被诊断出患有支气管哮喘。他对吸入皮质类固醇和长效β受体激动剂反应良好。发病大约18个月后,患者报告症状恶化。这些症状包括严重的功能限制,需要频繁接触高剂量泼尼松龙。Mepolizumab被添加到治疗中,导致支气管哮喘的最佳控制。尽管每月服用7剂美泊利单抗,患者出现颈部和耳后淋巴结病和皮下软组织肿胀。颈淋巴结活检证实了Kimura病的诊断。口服糖皮质激素和甲氨蝶呤治疗后,患者症状完全缓解。在过去的13个月中,他一直在缓解和口服泼尼松龙。在这种情况下,我们重点介绍了接受mepolizumab治疗的患者发生Kimura病的过程.
    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.
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  • 文章类型: Case Reports
    木村病(KD)是一种罕见的慢性炎症性疾病,其特征是皮下淋巴样增生伴周围嗜酸性粒细胞增多。据报道,15%-18%的KD成年患者肾脏受累,在许多情况下作为肾病综合征。我们介绍了一例与KD相关的重叠膜性肾病和IgA肾病。
    一名27岁的男子入院,在过去2个月有双侧腿部水肿的病史,并伴有宫颈肿块和发热的进行性增加。实验室检查结果如下:外周血白细胞计数,10,080/mmm3;嗜酸性粒细胞,3,200/mmm3(31.7%);血清肌酐,0.83mg/dL;和eGFR:每1.73m2140mL/min。尿液分析显示存在血尿和蛋白尿,结果如下:24小时蛋白尿,12.9g;血清白蛋白,1.3g/dL;IgE水平升高,750kU/L乙型肝炎血清学,丙型肝炎,艾滋病毒,VDRL均为阴性。补体C3和C4水平正常。在血液和尿液中未检测到单克隆蛋白。寄生虫侵扰被丢弃。颈淋巴结活检显示嗜酸性淋巴样增生,建议KD。肾活检显示发现与膜性肾病与IgA肾病的重叠一致。患者用泼尼松1mg/kg/d治疗KD,逐渐剂量逐渐减少,后结合甲氨蝶呤15mg/周。肾素-血管紧张素系统抑制剂用于肾病综合征。宫颈肿块消退,蛋白尿达到部分缓解,随着血清白蛋白水平的增加以及嗜酸性粒细胞和IgE水平的正常化。
    虽然不常见,KD患者必须考虑肾脏受累。肾小球疾病是最常见的肾损伤形式。
    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.
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  • 文章类型: Case Reports
    Kimura病(KD)是一种慢性炎症性疾病,其特征是累及头颈部的无触痛淋巴结病。KD的肾脏受累是罕见的,尤其是儿童。我们报告了一个12岁的男孩,该男孩先前曾接受过经典KD治疗,并在4年后出现了anasarca和少尿。没有肿胀或淋巴结肿大。肾活检显示膜性肾病。口服泼尼松龙和他克莫司治疗可缓解症状。该患者强调需要定期监测KD患者肾脏疾病的演变,即使淋巴结肿大消退.连续监测嗜酸性粒细胞增多,炎症标志物,需要尿液检查以帮助早期识别亚临床疾病并迅速开始特定治疗。
    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.
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  • 文章类型: Journal Article
    伴有嗜酸性粒细胞增多的血管淋巴样增生(ALHE)是一种罕见的疾病,其特征是主要在头颈部区域的结节性病变。经常引起不适或疼痛。由于治疗的稀有性和缺乏既定的指导方针,治疗仍然具有挑战性。本报告介绍了一例ALHE影响耳垂的病例,该病例通过耳小叶减少手术和随后的病灶内类固醇注射成功治疗。一名31岁的女性有复发性耳垂肿块的病史,接受了部分切除术,以避免耳垂丢失。组织病理学检查证实木村病,ALHE的变体。随后的局部甲基强的松龙注射有效控制了剩余的病变,导致在没有缺口的情况下显著的尺寸减小。对于这种情况,已经尝试了各种治疗方式;然而,复发率仍然很高。手术切除结合病灶内皮质类固醇注射是首选方法。在这种情况下,耳垂复位的耳垂下沟技术被用来保留耳小叶的外侧边缘,尽量减少畸形的风险,并实现可预测的结果。亚反耳沟技术提供了一种在不影响美观的情况下减小耳垂尺寸的方法。需要进一步的研究来阐明ALHE的发病机理,并建立针对这种罕见疾病的标准化治疗方案。
    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.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    木村病(KD)是一种罕见的,慢性,炎症状况,主要见于亚裔男性患者。它通常表现在50-60岁之间,通常患有双侧疾病。血管淋巴样增生伴嗜酸性粒细胞增多(ALHE)仍是主要的鉴别诊断,尽管组织学分析对于区分其他类似的病理至关重要。在这种情况下,我们提出了一个在中年人中的单侧眼眶KD的非典型病例,高加索人,男性绅士,没有区域淋巴结病的证据以及眼眶KD和鉴别诊断的文献综述,组织学特征和可用的管理方式,增加了关于该主题的稀疏文献。目前,没有公认的KD诊断标准,通过切开或切除活检进行组织病理学分析是主要的诊断方法。尽管管理正在转向保留类固醇的免疫调节疗法,但有或没有皮质类固醇管理的完全手术切除仍然是最常见的治疗方式。据我们所知,这是第一个描述使用霉酚酸酯维持治疗KD的病例。
    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.
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  • 文章类型: Journal Article
    木村病(KD)是一种罕见的慢性炎症性疾病,主要影响亚洲年轻男性,其特征是无痛性皮下肿块,淋巴结病,血清IgE水平升高。尽管它是良性的,KD由于其稀有性和临床变异性而提出了诊断和治疗挑战。
    本研究旨在通过回顾性病例系列对KD的临床和放射学特征进行全面分析,为了评估治疗结果,并讨论对诊断和管理的影响。
    我们回顾性分析了2018年1月至2023年10月浙江省人民医院收治的4例经组织学证实的KD病例。回顾性分析临床和放射学资料,和影像学发现由两名神经放射科医生分析,以确定病变特征和对比增强模式。
    我们的研究结果表明,患者主要是男性,平均年龄为43岁,年龄范围为13-71岁。所有患者均表现为无痛性皮下肿块,其中三人外周血嗜酸性粒细胞增多,血清IgE水平升高。射线照相,病变主要不明确,有异质性增强,伴有皮下脂肪萎缩。完整的手术切除和口服糖皮质激素是有效的治疗方法,随访期间未发现复发.
    在头颈部无痛性皮下肿块的鉴别诊断中应考虑KD,尤其是在嗜酸性粒细胞增多和IgE水平升高的情况下。我们的发现有助于了解KD的临床和放射谱,并强调由于复发风险需要长期随访。
    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.
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