儿童多系统炎症综合征(MIS-C)是一种罕见的发热性疾病,与2019年冠状病毒感染(COVID-19)相关的多系统器官受累,经常表现出模仿川崎病(KD)的特征,儿童的另一种高热症。MIS-C的发病机制和完整的临床谱知之甚少:目前尚不清楚MIS-C和KD是不同的综合征还是代表共同的谱。卡介苗(BCG)瘢痕的红斑和硬结是KD的特征性表现之一,对于婴儿期强制接种卡介苗的国家的诊断很有用。此外,在接种SARS-CoV-2疫苗后,在卡介苗瘢痕中也有这样的发现,这可能与分子模仿有关。然而,在MIS-C病例中,没有关于卡介苗瘢痕改变的报告.这里,我们报道了日本一名3岁西班牙裔男孩的MIS-C病例,卡介苗疤痕处有红斑和硬结。该患者在日本16月龄时接受了卡介苗接种。发病前四周,在家庭爆发后,他对SARS-CoV-2的聚合酶链反应(PCR)结果呈阳性,尽管他没有症状.他出现发烧和胃肠道症状,随后出现了完整KD的所有六个主要发现。他表现出充血性心力衰竭,静脉注射免疫球蛋白(IVIG)治疗后。他被诊断为MIS-C基于特征性的皮肤粘膜和胃肠道症状,心功能下降,和凝血病,除了与MIS-C一致的实验室数据BCG的发现从疾病的早期阶段就存在。患者对两种剂量的IVIG无效,第三个IVIG加泼尼松龙导致了退热和心力衰竭的改善。未观察到冠状动脉受累。这是第一例与伴有KD特征的MIS-C相关的BCG瘢痕处出现红斑和硬结,这可能会给临床和机械的见解在疾病的理解。由于MIS-C的全谱仍在发展,两者都是临床特征重叠的综合征,在婴儿期实施强制性BCG项目的国家,相对于KD具有KD特征的MIS-C的深度表型分析需要进一步的研究.
Multisystem Inflammatory Syndrome in Children (MIS-C) is a rare febrile disorder with multisystem organ involvement temporally associated with coronavirus 2019 infection (COVID-19) and frequently exhibits features mimicking Kawasaki disease (KD), another febrile disorder in children. The pathogenesis and the full clinical spectrum of MIS-C is poorly understood: It is still unclear whether MIS-C and KD are different syndromes or represent a common spectrum. The erythema and induration of Bacillus Calmette-Guérin (BCG) scar is one of the characteristic findings of KD, and is useful for the diagnosis in countries where BCG vaccination is mandated in infancy. Furthermore, such findings in BCG scar were also reported after SARS-CoV-2 vaccination, which may be related to molecular mimicry. However, there are no
reports of changes at the BCG scar in MIS-C cases. Here, we report a
case of MIS-C in a 3-year-old Hispanic boy in Japan, with erythema and induration at the BCG scar. The patient received BCG vaccination at 16 months of age in Japan. Four weeks before the onset, he had positive polymerase chain reaction (PCR) results for SARS-CoV-2 following household outbreak, although he was asymptomatic. He presented with fever and gastrointestinal symptoms, followed by the appearance of all six principal findings of complete KD. He exhibited congestive heart failure, following intravenous immunoglobulin (IVIG) therapy. He was diagnosed with MIS-C based on characteristic mucocutaneous and gastrointestinal symptoms, decreased cardiac function, and coagulopathy, in addition to laboratory data consistent with MIS-C. The BCG finding was present from the early stage of the disease. The patient was refractory to two doses of IVIGs, and the third IVIG plus prednisolone resulted in defervescence and improvement in heart failure. No coronary involvement was observed. This is the first
case of erythema and induration at the BCG scar associated with MIS-C accompanied by KD features, which may give clinical and mechanistic insights in the understanding of the disease. Since the full spectrum of MIS-C is still evolving and both of them are syndromes with overlapped clinical features, further studies are warranted for deep phenotyping of MIS-C with KD features relative to KD in countries with mandatory BCG programs in infancy.