Li Xiao-Lan, Luan Chun-Yan, Fan Ying-Jun, Lin Xiao-Ying, Jiang Dong, Su Mei-Xian, Wang Gang, Yang Xu
Department of Dermatology, The Second Affiliated Hospital of Kunming Medical University, Kunming, 650101, People's Republic of China.
Department of Rheumatology, The Second Affiliated Hospital of Kunming Medical University, Kunming, 650101, People's Republic of China.
J Inflamm Res. 2022 Jul 26;15:4251-4260. doi: 10.2147/JIR.S369986. eCollection 2022.
Purpura fulminans (PF), a rare, life-threatening disorder, is a hematological emergency in which there is skin necrosis, disseminated intravascular coagulation (DIC), and protein C deficiency. In PF, the skin necrosis and DIC are secondary to protein C deficiency. This may progress rapidly to multiorgan failure caused by the thrombotic occlusion of small- and medium-sized blood vessels.
This article presents the case of a 22-year-old male with fever as well as necrotic and purpuric skin lesions. The ultrasound and computed tomography scans revealed infections in the skin wounds as well as venous microthrombosis and thrombosis in multiple intracranial and pulmonary vessels. The laboratory tests showed signs of sepsis, thrombocytopenia, an abnormal decrease in protein C and antithrombin III, DIC, multiple organ and system failures, gastric varices, and gastrointestinal hemorrhage. The blood, sputum, and secretions under the skin lesions were cultured and were positive for . The results of the high-throughput genetic testing of the pathogenic microorganism DNA were consistent. In addition, human herpesvirus type 5 was detected. The histopathological examination of the skin lesions revealed pathological features consistent with PF. After successful treatment by the departments of Dermatology, Emergency Critical Care Medicine, and the Intensive Care Unit, the patient was discharged after 67 days of hospitalization.
Adults with acquired protein C and/or S deficiency states, including certain bacterial and viral infections, who drink alcohol and take varieties of non-steroidal anti-inflammatory analgesics at the same time, may develop acute infectious PF. Clinicians should be aware of this for early diagnosis and treatment.
暴发性紫癜（PF）是一种罕见的、危及生命的疾病，是一种血液学急症，其特征为皮肤坏死、弥散性血管内凝血（DIC）和蛋白C缺乏。在PF中，皮肤坏死和DIC继发于蛋白C缺乏。这可能迅速进展为中小血管血栓闭塞导致的多器官功能衰竭。
本文介绍了一名22岁男性患者，伴有发热以及坏死性和紫癜性皮肤病变。超声和计算机断层扫描显示皮肤伤口感染以及颅内和肺部多条血管的静脉微血栓形成和血栓形成。实验室检查显示有败血症、血小板减少、蛋白C和抗凝血酶III异常降低、DIC、多器官和系统功能衰竭、胃静脉曲张和胃肠道出血迹象。对血液、痰液和皮肤病变下的分泌物进行培养，结果呈阳性。致病微生物DNA的高通量基因检测结果一致。此外，检测到5型人类疱疹病毒。皮肤病变的组织病理学检查显示与PF一致的病理特征。经皮肤科、急诊重症医学科和重症监护病房成功治疗后，患者住院67天后出院。
患有获得性蛋白C和/或S缺乏状态的成年人，包括某些细菌和病毒感染，同时饮酒并服用多种非甾体类抗炎镇痛药，可能会发生急性感染性PF。临床医生应意识到这一点以便早期诊断和治疗。
