Tahghighi Fatemeh, Bakhtiari Koohsorkhi Maryam, Ziaee Vahid
Children's Medical Center, Pediatrics Center of Excellence, Tehran, Iran.
Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.
Case Rep Rheumatol. 2021 Apr 13;2021:6629405. doi: 10.1155/2021/6629405. eCollection 2021.
Diagnosing infantile Kawasaki disease with atypical symptoms is difficult, and it also has higher risk of coronary abnormalities which is one of the most common complications of KD. Other complications such as pericardial effusion, mitral insufficiency, congestive heart failure, myocardial systolic dysfunction, and systemic vasculitis were also reported. Peripheral gangrene and necrosis are among the rare complications of this systemic vasculitis. . We report an 8-month-old girl with prolonged fever, generalized petechial rash, cracked erythematous lips, edema, and coronary ectasia who received two doses of IVIG in another center, but short after her discharge, she started to develop a necrotic plaque on her knee. She was admitted in our hospital, and the repeat echocardiography showed sustained coronary ectasia. She received 3 doses of methylprednisolone pulse therapy and was discharged with aspirin and prednisolone. In the follow-up visits, the coronary ectasia was resolved and the necrotic ulcer was healing with a scar.
The diagnosis of Kawasaki disease and echocardiographic evaluation of the coronary arteries should be considered in young infants with prolonged fever of unknown origin. Peripheral gangrene is a rare but important complication of infantile Kawasaki disease, although the exact mechanism in not fully understood.
诊断具有非典型症状的婴儿川崎病较为困难，且其发生冠状动脉异常的风险较高，冠状动脉异常是川崎病最常见的并发症之一。还报道了其他并发症，如心包积液、二尖瓣关闭不全、充血性心力衰竭、心肌收缩功能障碍和系统性血管炎。外周坏疽和坏死是这种系统性血管炎的罕见并发症。我们报告一名8个月大的女孩，她持续发热、全身出现瘀点皮疹、嘴唇干裂发红、水肿且有冠状动脉扩张，在另一家中心接受了两剂静脉注射免疫球蛋白，但出院后不久，她的膝盖开始出现坏死性斑块。她入住我院，复查超声心动图显示冠状动脉持续扩张。她接受了3剂甲泼尼龙冲击治疗，出院时服用阿司匹林和泼尼松龙。在随访中，冠状动脉扩张得到缓解，坏死性溃疡正在愈合并留下疤痕。
对于不明原因长期发热的幼儿，应考虑川崎病的诊断及冠状动脉的超声心动图评估。外周坏疽是婴儿川崎病的一种罕见但重要的并发症，尽管其确切机制尚未完全明确。
