Ghosh Sudip Kumar, Bandyopadhyay Debabrata, Dutta Abhijit, Jane Esther P, Biswas Surajit Kumar
Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, Kolkata, India.
Department of Kolkata Medical College, Kolkata, India.
Indian J Dermatol. 2020 Sep-Oct;65(5):381-387. doi: 10.4103/ijd.IJD_8_19.
Purpura fulminans (PF) is a potentially fatal uncommon disorder of intravascular thrombosis and is clinically characterized by rapidly progressive hemorrhagic infarction of the skin.
To describe the clinical feature and outcome of a series of patients with PF.
A descriptive study based on review of case records was carried out at a tertiary care hospital in Kolkata.
Twenty three consecutive cases seen over a period of 8 years were studied. The age range was 4 days to 78 years (mean 35.6 years) with a male to female ratio of 1:2.8. Hemorrhagic rash was the universal presenting symptom. Other major presenting features included pneumonia (26.1%), sudden-onset shock syndrome (21.7%), and urinary tract infection (17.4%). All patients presented with retiform purpura and lesional necrosis and 8 (34.8%) patients had associated peripheral gangrene. Nineteen (82.6%) patients had sepsis and 60.9% patients had vesiculo-bullous lesion. was the most common (26.1%) pathogenic organism detected. The precise cause of PF could not be detected in two (8.7%) patients. One patient (4.3%) with neonatal PF had protein C deficiency. All patients had evidence of disseminated intravascular coagulation (DIC). One patient had to undergo a below knee surgical amputation and one patient had autoamputation of the digits. Ten (43.5%) patients succumbed to their illness. Seven of the 8 patients who had peripheral gangrene had a fatal outcome.
Relatively small sample size and a referral bias were a few limitations of the present study.
The present study emphasizes that PF is a cutaneous marker of DIC. Association of peripheral gangrene, leukopenia and neutropenia may be the reason for the high mortality rate.
暴发性紫癜（PF）是一种潜在致命的血管内血栓形成罕见疾病，临床特征为皮肤迅速进展性出血性梗死。
描述一系列PF患者的临床特征及预后。
在加尔各答的一家三级护理医院进行了一项基于病例记录回顾的描述性研究。
对8年期间连续观察的23例病例进行了研究。年龄范围为4天至78岁（平均35.6岁），男女比例为1:2.8。出血性皮疹是普遍的首发症状。其他主要首发特征包括肺炎（26.1%）、突发休克综合征（21.7%）和尿路感染（17.4%）。所有患者均出现网状紫癜和皮损坏死，8例（34.8%）患者伴有外周坏疽。19例（82.6%）患者发生败血症，60.9%患者有水泡大疱性皮损。 是检测到的最常见（26.1%）致病微生物。2例（8.7%）患者未检测到PF的确切病因。1例新生儿PF患者（4.3%）有蛋白C缺乏症。所有患者均有弥散性血管内凝血（DIC）证据。1例患者不得不接受膝下手术截肢，1例患者手指自动截肢。10例（43.5%）患者因病死亡。8例有外周坏疽的患者中有7例预后不良。
本研究的一些局限性为样本量相对较小和转诊偏倚。
本研究强调PF是DIC的皮肤标志物。外周坏疽、白细胞减少和中性粒细胞减少的关联可能是高死亡率的原因。
