本病罕见,常不易诊断。其临床和病理与典型的或成人结节性多动脉炎不同,但与致死性粘膜皮肤淋巴结综合征(MLNS)相同,常由于冠状动脉受累而突然死亡。本文报告一例,4月男婴,因发热、呕吐和腹胀2天而入院。检查有眼结合膜炎,口腔和咽部粘膜弥漫性发红,哭声嘶哑,有干咳,但肺部清晰。心血管系统正常。腹部膨胀。入院后体温波动在38～40℃之间,大便稀薄,数日后正常。入院第4天皮肤起红斑,四肢开始,扩展至躯干并相互融合,皮疹数小时后消退。但次日又出现,发病第12天指趾端脱屑。持续性心动过速,但无其他心力衰竭症状。血压正常。第14天死亡。发热原因未找到,血、粪、尿培养均阴性。LE细胞未找到。CSF正常。胸腹X线摄片阴性。心电图示窦性心动过速。虽无明显感染,但因发热及白细胞数增高, The rare disease, often not easy to diagnose. The clinical and pathological and classic or adult polyarteritis nodosa different, but with the same deadly mucous membrane cutaneous lymph node syndrome (MLNS), often due to coronary artery involvement and sudden death. This article reports an example of a baby boy who was admitted to hospital in April due to fever, vomiting and bloating for 2 days. Check eyes with conjunctivitis, oral and pharyngeal mucosa diffuse redness, crying hoarseness, dry cough, but the lungs clear. Cardiovascular system is normal. Abdominal swelling. After admission, body temperature fluctuations between 38 ~ 40 ℃, stool thin, a few days later normal. On the 4th day of admission, the skin developed erythema, the limbs began to extend to the trunk and merge with each other, and the rash subsided after a few hours. But the next day it appeared, the first 12 days of disease refers to the tip of the desquamation. Persistent tachycardia, but no other heart failure symptoms. Normal blood pressure. Died on the 14th day. Fever not found, blood, urine, urine culture were negative. LE cells not found. CSF is normal. Thoracoabdominal X-ray negative. Electrocardiogram shows sinus tachycardia. Although no significant infection, but due to fever and increased white blood cell count,