Mai Yumiao, Wang Yingjie, Sun Pan, Jing Zhaohe, Dong Pengpeng, Liu Jian
Department of Pediatrics, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Front Pediatr. 2023 Feb 17;11:1131963. doi: 10.3389/fped.2023.1131963. eCollection 2023.
Kimura disease (KD), also known as eosinophilic hyperplastic lymphoid granuloma, is a rare benign chronic inflammatory condition, which is featured with the painless progressive mass located in the subcutaneous area of the head and neck region, elevated peripheral blood eosinophils, and raised serum immunoglobulin E (IgE) levels. KD is uncommon in clinical practice, especially with low incidence in children, so it often leads to misdiagnosis or missed diagnosis in pediatric patients.
The clinical data of 11 pediatric patients with KD in the authors' institution were retrospectively analyzed.
There were 11 pediatric patients with KD enrolled in total, including 9 male patients and 2 female patients (sex ratio 4.5:1). The median age at diagnosis stood at 14 years (range 5-18 years), the initial symptoms in all patients included painless subcutaneous masses and focal swelling, the duration of symptoms ranged from 1 month to 10 years, and the average duration was 24.45 months. Six patients had single lesions, and 5 had multiple lesions. The highest proportion of lesion regions were parotid gland ( = 5, 31.3%) and retroauricular ( = 5, 31.3%), followed by cervical lymph nodes ( = 4, 25%), and others ( = 2,12.5; elbow = 1; back = 1). The eosinophil absolute count elevated in all patients, ranging from 0.71×10/L to 10.35 ×10/L (normal range 0.02-0.52×10/L). IgE levels were increased in all 7 patients who underwent serum immunoglobulin examination (normal range <100 IU/mL). Three patients received oral corticosteroid treatment while 2 relapsed. Three patients received surgical resection combined with oral corticosteroid treatment, and no patient relapsed. The other 3 patients received surgery and radiotherapy, surgery combined with corticosteroid and cyclosporin and corticosteroid combined with leflunomide respectively, and no patient relapsed.
Base on the study, it is found that Kimura disease is rare and may have the atypical symptoms in pediatric patients, combination therapy is recommended to reduce recurrence, and long-term follow-up should be performed.
木村病（KD），又称嗜酸性淋巴肉芽肿，是一种罕见的良性慢性炎症性疾病，其特征为头颈部皮下无痛性进行性肿块、外周血嗜酸性粒细胞增多以及血清免疫球蛋白E（IgE）水平升高。KD在临床实践中并不常见，尤其是在儿童中发病率较低，因此在儿科患者中常导致误诊或漏诊。
回顾性分析作者所在机构11例儿童KD患者的临床资料。
共纳入11例儿童KD患者，其中男性9例，女性2例（性别比4.5:1）。诊断时的中位年龄为14岁（范围5 - 18岁），所有患者的初始症状均包括无痛性皮下肿块和局部肿胀，症状持续时间为1个月至10年，平均持续时间为24.45个月。6例患者为单发病变，5例为多发病变。病变区域比例最高的是腮腺（=5，31.3%）和耳后（=5，31.3%），其次是颈部淋巴结（=4，25%），其他部位（=2，12.5；肘部=1；背部=1）。所有患者嗜酸性粒细胞绝对计数均升高，范围为0.71×10⁹/L至10.35×10⁹/L（正常范围0.02 - 0.52×10⁹/L）。接受血清免疫球蛋白检查的7例患者IgE水平均升高（正常范围<100 IU/mL）。3例患者接受口服糖皮质激素治疗，2例复发。3例患者接受手术切除联合口服糖皮质激素治疗，无患者复发。另外3例患者分别接受手术和放疗、手术联合糖皮质激素和环孢素以及糖皮质激素联合来氟米特治疗，无患者复发。
基于本研究发现，木村病在儿科患者中罕见且可能有非典型症状，建议采用联合治疗以减少复发，并应进行长期随访。
