Takayanagi K, Grochwska E, Al-Bader M, Doresowamy N, Freeman N V
Z Kinderchir. 1985 Jun;40(3):182-7. doi: 10.1055/s-2008-1059741.
Three cases of purpura fulminans are reported. A five-year-old boy was successfully treated for purpura fulminans but had autoamputation of both legs and autolysis of bladder and urethra. He did not show any sign of shock at the onset of the disease. However, before an irreversible change of the skin lesion and organ involvement occurred, prompt diagnosis was effected, and aggressive treatment was given. An eight-day-old girl suffered from purpura fulminans at her four extremities, skull, bladder, ovary and vagina. Interesting findings were obtained in case 2, who had a positive family history. Several black spots in the extremities had disappeared spontaneously before admission, whereas the other lesions got progressively worse, with subsequent severe attack. Her general condition was not parallel to the severity of the disease. A 20-day-old boy had purpura fulminans after severe aspiration pneumonia, and showed a typical clinical course of the disease. In all patients, sudden drop of Hb and leukocytosis occurred after the skin lesion appeared. The diagnosis was unequivocal in these cases. Clotting time and FDP were good indicators of the progress of the disease in conjunction with the clinical features. Prompt diagnosis and aggressive therapy are necessary, not according to the patient's condition, but according to the laboratory findings and the serious nature of the disease. Internal haemorrhage must be considered as well as the skin lesion, this being a fatal complication.
报告了3例暴发性紫癜。一名5岁男孩成功治疗了暴发性紫癜，但双腿发生了自截，膀胱和尿道发生了自溶。他在疾病发作时没有任何休克迹象。然而，在皮肤病变和器官受累出现不可逆变化之前，进行了及时诊断并给予了积极治疗。一名8天大的女孩四肢、颅骨、膀胱、卵巢和阴道均出现暴发性紫癜。在病例2中获得了有趣的发现，该病例有家族史阳性。入院前四肢的几个黑点已自行消失，而其他病变则逐渐恶化，随后发生严重发作。她的一般状况与疾病的严重程度不平行。一名20天大的男孩在严重吸入性肺炎后发生暴发性紫癜，并表现出该疾病的典型临床病程。所有患者在皮肤病变出现后均出现血红蛋白突然下降和白细胞增多。这些病例的诊断明确。凝血时间和纤维蛋白降解产物结合临床特征是疾病进展的良好指标。必须进行及时诊断和积极治疗，不是根据患者的状况，而是根据实验室检查结果和疾病的严重性质。必须考虑内出血以及皮肤病变，这是一种致命的并发症。
