IntroductIonSymmetrical peripheral gangrene (SPG) is a rare complication in the intensive care unit.Its definition involves symmetrical ischemic damage, which occurs at the distal part of the limbs or genitalia, in the absence of a major vascular occlusive disease.It has high risk of mortality and high frequency of multiple limbs amputation in those who survive.[1] SPG should be suspected at the first sign of marked coldness, pallor, cyanosis, or pain in an extremity as the condition can progress rapidly to acrocyanosis and if not reversed frank gangrene.SPG is also sometimes referred to as purpura fulminans (PF).PF is an acute thrombotic disorder which manifests as blood spots, bruising, and discoloration of the skin.It results from coagulation in small blood vessels within the skin and rapidly leads to skin necrosis and disseminated intravascular coagulation (DIC).[2] PF is rare and most commonly occurs in babies and small children although it can also occur in adults with severe sepsis.Its presence is almost always a marker of DIC.[3] In Warkentin's review, both are of the same pathologic condition, i.e., microthrombosis, and both are of the same spectrum of manifestations.When there is additional or predominant nonacral tissue necrosis, the term PF is applicable.Otherwise, it is defined as SPG for clinical investigations. [4]4] etIology The etiology of SPG is multifactorial; however, in general, it has been divided into two main categories which are infective factors and noninfective factors.[5][6][7][8][9] Infective etiology of SPG is as follows: Pneumococcus, [10,11] Staphylococcus aureus, [10,12] Neisseria meningitidis, [7,12] Streptococcus pyogenes, [10] Klebsiella pneumoniae, Escherichia coli, Salmonella paratyphi, Proteus vulgaris, Proteus mirabilis, Pasteurella multocida, [13] Pseudomonas, [10,12] Enterococcus faecalis, [14] Capnocytophaga canimorsus (DF2), [15] Plasmodium falciparum, [16] Mycobacterium tuberculosis, [17] Rubeola, Varicella zoster, [13,18] dengue, [19] and human immunodeficiency virus.[20] Noninfective etiology of SPG refers to conditions that precipitate SPG occurrence such as myocardial infarction, [13] cardiac failure, [13,18] hypovolemic shock, [18] hypertension, [18] pulmonary embolism, [13] supraventricular t a c h y c a r d i a , [ 2 1 ] H o d g k i n 's l y m p h o m a , [ 2 2 ] l u n g adenocarcinoma, [23] adenocarcinoma-associated thrombotic Symmetrical peripheral gangrene (SPG) is a rare, debilitating disease that deserves more widespread concern among the medical fraternities.The objective of this review is to outline the etiology, pathology findings, and management practices of SPG.About 18%-40% mortality rate was reported, and survivors have high frequency of multiple limb amputations.SPG is the hallmark of disseminated intravascular coagulation (DIC).The main pathogenesis theory, to date, is microthrombosis associated with disturbed procoagulant-anticoagulant balance.The treatment of SPG is largely anecdotal and theoretically involves heparin-based anticoagulation and substitution of natural anticoagulants.Early recognition, prompt management of DIC, and underlying conditions may halt the progression of the disease.The multicenter randomized controlled trial should be set up to formulate the proper treatment guidelines.
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