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血管加压素诱导的暴发性紫癜的罕见表现。

An Uncommon Presentation of Vasopressin-Induced Purpura Fulminans.

作者信息

Awad Vanessa, Nair Preeth, Roy Sasmit, Yalamanchili Anish, Adapa Sreedhar, Vemuri Nirupama

机构信息

Department of Internal Medicine, Sierra View Medical Center, Porterville, CA, USA.

Department of Internal Medicine, University of Virginia, Charlottesville, VA, USA.

出版信息

J Med Cases. 2023 Apr;14(4):130-136. doi: 10.14740/jmc4062. Epub 2023 Apr 30.

DOI:10.14740/jmc4062
PMID:37188301
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10181292/
Abstract

Purpura fulminans (PF) is a rarely encountered rapidly evolving dermatological manifestation of ischemia, particularly in critically ill patients. Considered one of the very few dermatological emergencies, it has high mortality rate where patients often succumb to the illness. It can manifest in three forms: neonatal, idiopathic, and the more commonly infectious variety, which can be secondary to mostly bacterial and rarely viral etiology. It is also reported to be highly associated with disseminated intravascular coagulation (DIC), heparin-induced thrombocytopenia (HIT), and acute hepatic failure (AHF). Hereditary or acquired deficiency of protein C and dysregulation of the coagulation cascade, mainly protein C-thrombomodulin, has been implicated in the pathogenesis. We present a 55-year-old male admitted to the intensive care unit for diabetic ketoacidosis (DKA) and septic shock. Along with initiating management protocol for DKA and broad-spectrum antibiotics, he was initially started on norepinephrine for septic shock. Because of persistent refractory septic shock, he was subsequently initiated on phenylephrine and vasopressin to maintain adequate perfusion. The following day, he was found to have sharply demarcated blackish non-blanching discoloration on bilateral knees, lower limbs, and scrotum, sparing the acral regions. This cutaneous manifestation persisted throughout his hospital course, although it improved after discontinuation of vasopressin while continuing with other pressors. Vasopressin has been implicated in a few instances of skin necrosis; however, PF has rarely been documented and never within 1 day like ours. This case demonstrates a unique development of PF likely from vasopressin after ruling out the diagnoses of DIC, HIT, thrombotic thrombocytopenic purpura, and AHF.

摘要

暴发性紫癜(PF)是一种罕见的、迅速发展的缺血性皮肤病表现,尤其在危重症患者中。它被认为是极少数的皮肤科急症之一,死亡率很高,患者常因此病死亡。它可表现为三种形式:新生儿型、特发型和更常见的感染型,后者大多继发于细菌病因,很少继发于病毒病因。据报道,它还与弥散性血管内凝血(DIC)、肝素诱导的血小板减少症(HIT)和急性肝衰竭(AHF)高度相关。遗传性或获得性蛋白C缺乏以及凝血级联反应失调,主要是蛋白C - 血栓调节蛋白失调,被认为与发病机制有关。我们报告一例55岁男性,因糖尿病酮症酸中毒(DKA)和感染性休克入住重症监护病房。在启动DKA管理方案和使用广谱抗生素的同时,最初他因感染性休克开始使用去甲肾上腺素。由于持续性难治性感染性休克,随后他开始使用去氧肾上腺素和血管加压素以维持足够的灌注。第二天,发现他双侧膝盖、下肢和阴囊出现边界清晰的黑色非压之褪色的变色,肢端未受累。这种皮肤表现贯穿他的住院病程,尽管在停用血管加压素而继续使用其他升压药后有所改善。血管加压素曾在一些皮肤坏死病例中被牵连;然而,PF很少有文献记载,且从未像我们的病例这样在1天内出现。该病例排除了DIC、HIT、血栓性血小板减少性紫癜和AHF的诊断后,显示了可能由血管加压素导致的PF的独特发展过程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/804613efd6c4/jmc-14-130-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/8350d8c2eb2a/jmc-14-130-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/73c0eec97abe/jmc-14-130-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/804613efd6c4/jmc-14-130-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/8350d8c2eb2a/jmc-14-130-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/73c0eec97abe/jmc-14-130-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6b6/10181292/804613efd6c4/jmc-14-130-g003.jpg

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