背景:井综合征,或者嗜酸性细胞织炎,在童年是罕见的,自1979年以来,报告的儿科病例不到40例。病理生理学未知。
方法:2012年2月,儿科皮肤病学会研究小组成员提交了0-15岁儿童的Wells综合征病例。临床细节,医师使用标准化问卷收集生物学和组织学特征以及治疗策略.图片由作者审查。
结果:包括11名患者(平均年龄:6岁),有很强的特应性患病率(63%)。注意到两种类型的临床表现:单发或多发蜂窝织炎,伴有或不伴有膀胱小叶病变和固定荨麻疹。82%的患者有瘙痒,73%的患者有嗜酸性粒细胞增多。对于所有患者来说,皮肤活检的组织学检查显示,嗜酸性粒细胞浸润在真皮中延伸,并在2例患者中观察到相关的Sweet样嗜中性粒细胞浸润。病程延长(平均持续时间:8个月),伴有发作。治疗方法因医生而异(局部或全身类固醇,他克莫司和氨苯砜)。
结论:我们的研究证实了文献中有关临床,儿童Well\'s综合征的组织学特征和病程。关键信息是迄今为止未报告的特应性儿童的高患病率。在昆虫叮咬的环境中,疫苗接种,感染或创伤,这种不寻常的背景可以解释嗜酸性粒细胞炎症反应的发生.必要时,口服或局部类固醇似乎是儿童的一线治疗。
结论:Well\'s综合征在儿童中是罕见的,并以其多态性为特征。我们首次报道了一系列患者中特应症的高患病率,这为理解这些罕见疾病提供了新的视角。我们建议局部使用类固醇作为浅表病变儿童的一线治疗,口服类固醇用于治疗纤维素病变或局部治疗失败。
BACKGROUND: Well\'s syndrome, or eosinophilic cellulitis, is rare in childhood, with fewer than 40 pediatric cases being reported since 1979. The physiopathology is unknown.
METHODS: In February 2012, members of the research group of the Department of Pediatric Dermatology Society submitted their case of Wells\' syndrome in children aged 0-15 years. Details of clinical, biological and histological features and of therapeutic strategies were collected by physicians using a standardized questionnaire. Pictures were reviewed by the authors.
RESULTS: Eleven patients were included (average age: 6 years), with a strong prevalence of atopy (63%). Two types of clinical manifestation were noted: single or multiple cellulitis associated or not with vesiculobullous lesions and fixed urticaria. Eighty-two percent of patients had pruritus and 73% had eosinophilia. For all patients, histological examination of skin biopsies showed an eosinophilic infiltrate extending in the dermis with associated Sweet-like neutrophilic infiltrate being seen in 2 patients. The course of the disease was protracted (mean duration: 8 months) with flare-ups. Treatment varied depending on the doctors (topical or systemic steroids, tacrolimus and dapsone).
CONCLUSIONS: Our study confirms some of the data in the literature concerning the clinical, histological features and course of Well\'s syndrome in children. The key information is the high prevalence of atopic children hitherto unreported. In a setting of insect bites, vaccination, infection or traumatism, this unusual background could explain the onset of inflammatory reaction with eosinophils. Oral or topical steroids appear to be the first-line treatment in children when necessary.
CONCLUSIONS: Well\'s syndrome in children is rare and characterized by its polymorphism. We report for the first time in a series of patients a high prevalence of atopy, which raises new perspectives in understanding these rare diseases. We propose topical steroids as first-line therapy in children with superficial lesions, with oral steroids being given for cellulitic lesions or where topical therapy fails.