|Year : 2021 | Volume
| Issue : 3 | Page : 168-170
Symmetrical peripheral gangrene: A rare clinical syndrome
Ashok Kumar, Urvashi Khan, Shivani Bansal
Department of Medicine, Santosh Medical Dental College and Hospital, Ghaziabad, Uttar Pradesh, India
|Date of Submission||09-Feb-2021|
|Date of Decision||13-Feb-2021|
|Date of Acceptance||13-Feb-2021|
|Date of Web Publication||30-Jul-2021|
Dr. Ashok Kumar
Department of Medicine, Santosh Medical Dental College and Hospital, No. 1, Ambedkar Road, Old Bus Stand, Ghaziabad - 201 001, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Symmetrical peripheral gangrene (SPG) is a rare syndrome which is defined by the peripheral ischemic lesion of two or more extremities in the absence of major vascular obstructive disease. We hereby describe a case of 63-year-old female, admitted in intensive care unit with a history of shortness of breath with atrial fibrillation since few days, developed cold extremity with acrocyanosis that rapidly progressed to gangrene. Laboratory analysis revealed increased inflammatory parameters, deranged liver enzymes, thrombocytopenia, and prolonged coagulation time. Patient developed septic shock and was started on intravenous vasopressor with clinical benefits. Even though there is no consensus regarding SPG treatment, consequences should be mitigated, particularly when vasopressor are used, in order to avoid major amputation. Most of the sepsis survivors with SPG require amputation of the affected extremities.
Keywords: Septic shock, symmetrical peripheral gangrene, vasopressor
|How to cite this article:|
Kumar A, Khan U, Bansal S. Symmetrical peripheral gangrene: A rare clinical syndrome. Indian J Med Spec 2021;12:168-70
|How to cite this URL:|
Kumar A, Khan U, Bansal S. Symmetrical peripheral gangrene: A rare clinical syndrome. Indian J Med Spec [serial online] 2021 [cited 2021 Sep 19];12:168-70. Available from: http://www.ijms.in/text.asp?2021/12/3/168/322781
| Introduction|| |
Symmetrical peripheral gangrene (SPG) is a very rare devastating entity which is a severe complication of disseminated intravascular coagulation (DIC) that is usually associated with sepsis. It was described for the first time in 1891 by Hutchinson in a clinical case of sepsis with intravascular disseminated coagulation. It is characterized by symmetric necrosis of the skin and distal extremities, following which two or more distal sites become gangrenous in the absence of large artery occlusion. It is difficult to prevent the development of gangrene and amputation cannot be avoided in SPG; however, a correct diagnosis and appropriate surgical procedure are required to improve patient's quality of life. We should consider other differential diagnosis such as frostbite, vasospasm (scleroderma associated Raynaud's phenomenon), calciphylaxis, myeloproliferative, or lymphoproliferative disorders (including monoclonal gammopathies), vasculitis, rheumatologic or immunologic disorders, antiphospholipid syndrome and uncontrolled proinflammatory disorders such as ulcerative colitis.
| Case Report|| |
A 63-year-old female presented at the emergency department with sudden onset shortness of breath and palpitations in the previous 48–72 h. The patient was admitted to the intensive care unit (ICU) with the diagnosis of atrial fibrillation with septic shock secondary to Pneumonitis. COVID RTPCR was negative for the patient. Laboratory investigations indicated initially normal to borderline increased leukocytes (10220/mm3), CRP of 95 mg/dl and no renal or hepatic failure. Arterial blood gases (ABG) revealed metabolic acidosis (ph 7.215), increased lactates (4.16 mmol/l), and low bicarbonates (13.0 meq/l). After few hours of admission, the patient developed cold extremities with acrocyanosis of the right hand and both feet. The lesions rapidly evolved to gangrene [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f. On examination, patient was conscious, oriented, her SpO2 was 50%–70%, tachypnea was present (RR-26/min), peripheral pulses were not palpable, blood pressure 70/40 mmHg with cold peripheries, and bluish discoloration of extremities was present, Jugular Venous Pressure was raised. Her chest X-ray (CXR) was suggestive of bilateral parahilar opacity and electrocardiography showed atrial fibrillation, fast ventricular response with coved ST depression in anterior leads suggestive of myocardial ischemia. Her two-dimensional echocardiography showed regional wall motion abnormalties in the anterior wall with EF-30% with atrial fibrillation. She was managed with, broad-spectrum antibiotics, noninvasive ventilation BiPAP, vasopressor, injection amiodarone infusion with supportive measures. Despite fluid resuscitation, she needed high doses of noradrenaline to maintain blood pressure.
|Figure 1: Extent of involvement of gangrene in peripheral extremities. (a) Ventral aspect of the right hand (b) dorsum of the right hand (c) dorsum of left hand middle finger (d) ventral aspect of left hand middle finger (e) and (f) right and left foot, respectively|
Click here to view
Her laboratory investigations showed increased inflammatory parameters (procalcitonin-1.2 ng/ml), acute renal injury, thrombocytopenia, markedly elevated hepatic transaminase levels, and prolongation of coagulation times (prothrombin time-65.8 and international normalized ratio-4.87), revealing intravascular disseminated coagulation. Over the period of 2–3 days of ICU stay patient started showing signs of improvement, requirement of inotropes decreased, dyspnea and urine output improved. By the 4th day all the vasopressor supports were weaned off. However, cyanosis of the extremities was noted, small blebs and blisters were noticed in the right hand which progressed to gangrene formation over the next 1 week. [Figure 1] shows the extent of involvement of SPG in the upper and lower extremities. In the chest X-ray anteroposterior view of patient, there was bilateral parahilar opacity suggestive of acute pulmonary edema [Figure 2]. Doppler evaluation revealed the presence of diminished pulsations in all the vessels supplying the upper limbs and no flow in distal radial and ulnar artery as well as a superficial venous thrombosis in the basilic vein. [Table 1] shows the serial laboratory investigation in the patient. Later amelioration of the cyanotic areas in the foot was observed. A diagnosis of atrial fibrillation with fast ventricular response with septic shock with Multi Organ Dysfunction Syndrome, disseminated intravascular coagulation with SPG was made.
| Discussion|| |
There have been very few case reports and not much literature found on SPG. Purpura fulminans term is used occasionally synonymously but it is observed rather acute, quickly progressive purpuric skin lesions. The rapidly progressive lesion in purpura fulminans leads to necrosis of skin, organ dysfunction along with gangrenous changes in limbs. SPG usually presents with ischemic limb injury, sharply demarcated and striking symmetry that progress rapidly to necrosis.
Typically, they are associated with thrombocytopenia and coagulopathy in critically ill patients. Metabolic acidosis and acute ischemic hepatitis may be present, with the onset of limb ischemic necrosis beginning 2–5 days after the initial increase in liver enzymes as we could also relate in our case.
The pathogenesis of SPG is not very well explained. Although it has been understood through various case reports and cases series that it is related to the conditions with decreased blood supply and delivery of oxygen and nutrients to peripheral regions for an extended period. Decreased blood flow along with DIC seems to be implicating cause in most cases. Microcirculatory alterations, vasospastic conditions, such as the use of vasoactive drugs, and states of low cardiac output are considered as the most frequent associations with SPG.
Possible etiological factors cited in the literature include obstructive intracardiac lesions, sepsis, vasospastic conditions, small vessel obstruction, protein C deficiency, use of vasopressor agents, low cardiac output states and DIC. It is well established that the digital perfusion will drop to zero in the presence of persistently low perfusion pressures of 35–60 mmHg.
We describe a case in which multiple factors played an important role in the development of SPG such as septic shock, atrial fibrillation, and the use of vasopressor noradrenaline. The hypotension and vasopressor therapy reduced blood flow into the distal extremities, and therefore predispose the patient with septic shock with DIC to microthrombosis and consequent ischemia and progression to necrosis and gangrene. SPG associated with DIC has a mortality rate of approximately 35%, nevertheless, about 50% of survivors require amputations of at least one limb. After the patient's general condition improved and the lesions are demarcated, the patient was referred to surgery for the management of the gangrenous area.
| Conclusion|| |
In septic shock, there is a derangement affecting all organ systems including coagulation and microcirculation, resulting in hypoperfusion to peripheries. Awareness, early recognition, and prompt management of this condition are necessary to avoid catastrophes such as SPG. When the patient needs to be on vasopressors and inotropes, care should be taken to optimize the fluid. Vasopressor therapy should be withdrawn as early as possible, especially in the presence of any coldness and discoloration in the extremity.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Warkentin TE. Ischemic limb gangrene with pulses. N Engl J Med 2015;373:642-55.
Agha RA, Fowler AJ, Saeta A, Barai I, Rajmohan S, Orgill DP, et al
. The SCARE statement: Consensus-based surgical case report guidelines. Int J Surg 2016;34:180-6.
Tripathy S, Rath B. Symmetric peripheral gangrene: Catch it early! J Emerg Trauma Shock 2010;3:189-90.
Ghosh S, Bandyopadhyay D, Ghosh A. Symmetrical peripheral gangrene: A prospective study of 14 consecutive cases in a tertiary-care hospital in eastern India. J Eur Acad Dermatol Venereol 2010;24:214-8.
Sharma BD, Kabra SR, Gupta B. Symmetrical peripheral gangrene. Trop Doct 2004;34:2-4.
Shenoy R, Agarwal N, Goneppanavar U, Shenoy A, Sharma A. Symmetrical peripheral gangrene – A case report and brief review. Indian J Surg 2013;75:163-5.
[Figure 1], [Figure 2]