Adult Purpura Fulminans and Digital Necrosis Associated With Sepsis and the Factor V Mutation

Jackson, R. T.

doi:10.1001/jama.280.21.1829

Cited by 15 publications

(3 citation statements)

References 4 publications

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“…The presence of the FV G1691A mutation in six (46%) of 13 small children indicates that this mutation may be a risk factor for PF in children of a young age, as has been shown previously [4,12,14]. The association between post-infectious PF and FV G1691A mutation has also been reported previously [4,12,14,19]. In these studies, the need for an evaluation of hereditary defects in the inhibitory mechanisms of blood coagulation in patients with PF has been emphasized [4,12,14].…”

Section: Discussionsupporting

confidence: 54%

Outcome in children with purpura fulminans: Report on 16 patients

et al. 2005

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Purpura fulminans (PF) is a severe disorder of acute onset with high morbidity and mortality. In children, this rapidly progressive illness is usually associated with severe bacterial or viral infections. However, some other conditions may participate in the development of PF. Our objective was to investigate the underlying and associated disorders and the outcomes of the disease in 16 children, 7 males and 9 females ranging in age from 3.5 months to 12 years (median age, 2 years). Thirteen of the 16 children (81%) were 4 years of age or younger. The remaining 3 patients were 9, 10, and 12 years of age. Among these 13 infants and small children, 7 (43%) had infection, 2 infants had congenital cardiac disorders necessitating minor or major surgical intervention, and 1 infant and 3 children had different miscellaneous disorders. The factor V G1691A mutation was present in six of the 13 small children (46%). None of the 3 older children carried the mutation. Six (37.5%) of the 16 patients had protein C deficiencies, and 9 (56%) had protein S deficiencies. These deficiencies, except one for protein S, were acquired. Ten patients except two who were diagnosed at this center were treated with fresh frozen plasma. They were also given heparin. Nine (69%) of the 13 children 4 years of age or younger and one of the older children (33%) required amputation. Five of the six patients (83%) who had factor V G1691A mutation, and who also exhibited severe infection, required amputation. This study suggests that an age of 4 years or less is a risk factor for the development of PF during severe infections, especially in the presence of factor V G1691A mutation and congenital heart disease, necessitating major or minor surgical interventions. This study also shows that the amputation rate in 10 patients, after excluding the patients who had been referred to our center after development of sequelae, was 60%. The survival rate among these 10 patients may indicate that, with the treatment protocol, PF need not be regarded as a lethal disease any more. It is also suggested that effective immunization programs and better health care have probably resulted in some changes in the etiological profile of PF. Am. J. Hematol. 80:20–25, 2005. © 2005 Wiley‐Liss, Inc.

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Section: Discussionsupporting

confidence: 54%

Outcome in children with purpura fulminans: Report on 16 patients

et al. 2005

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“…To date there have been eight reported cases of purpura fulminans associated with factor V Leiden mutation, six children and two adults. 2–7 In only one case was there a homozygous factor V Leiden mutation. 6 The remaining five childhood and two adult cases were associated with a heterozygous factor V Leiden mutation.…”

Section: Discussionmentioning

confidence: 99%

Severe digital necrosis in an elderly patient with heterozygous factor V Leiden mutation

Patel

Morris²,

Rashid

et al. 2000

British Journal of Dermatology

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We report an 81-year-old female with a heterozygous factor V Leiden mutation who developed purpura fulminans. Digital necrosis, a characteristic clinical feature of purpura fulminans was prominent. Purpura fulminans is more common in children and adult cases are rare. Of eight reported cases of purpura fulminans resulting from a heterozygous factor V Leiden mutation recorded in the literature, only two were in adults: 40 and 42 years of age, respectively. This is the first report of this condition arising in a patient in her eighties.

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“…Symmetrical peripheral gangrene is described as multiple extremity ischemia at two or more sites in the absence of large vessel obstruction [1,2]. Possible etiological factors cited in literature include obstructive intracardiac lesions [3], sepsis [4], vasospastic conditions [5,6], small vessel obstruction [1], protein C deficiency [7], use of vasopressor agents [8,9], low cardiac output states [10,11], disseminated intravascular coagulation (DIC) [12,13], factor V Leiden mutation [14], and parenteral abuse of the sublingually available form of buprenorphine [15]. It is well established that the digital perfusion will drop to zero in presence of persistently low perfusion pressures of 35-60 mmHg [16].…”

Section: Discussionmentioning

confidence: 99%

Symmetrical Peripheral Gangrene—A Case Report and Brief Review

et al. 2012

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A 30 year-old gentleman presented to casualty with history of pain abdomen for six days, fever and decreased urine output since two days. He was in a state of septic shock and was diagnosed to have intestinal perforation. His peripheral pulses were not palpable except for the femoral and brachial vessels. Despite fluid resuscitation, he needed infusion of high doses of dopamine and noradrenaline to maintain his blood pressure. He was operated for repair of perforation. On the first postoperative day, in the intensive care unit, vasopressin infusion was added in view of persistent hypotension. Appropriate fluid resuscitation and antibiotic therapy helped to wean him off inotropes and vasopressors by the second postoperative day. On the 3rd postoperative day, however, the patient developed discolouration and blebs on the fingers of left hand, followed by the right hand and then both the lower limbs. Subsequently, over a period of 10 days, this progressed to gangrene formation in the hands despite the patient being haemodynamically stable without any inotropes or vasopressors in this period. We conclude that the septic shock is a systemic derangement affecting all organ systems including coagulation and microcirculation. Early recognition and prompt management of sepsis, optimisation of fluid status to wean off the inotropes and vasopressors at the earliest is necessary to avoid catastrophes such as symmetrical peripheral gangrene.Keywords Symmetrical peripheral gangrene . Sepsis . Vasopressors . Multiple arterial punctures . Resuscitation. Case ReportA 30-year-old man, an agriculturist by occupation, presented to the casualty ward with abdominal pain for 6 days and fever and reduced urine output for 2 days. He was in a state of intense septic shock with absent peripheral pulsations, and the pulse oximeter could not pick up pulsations. Despite fluid resuscitation, he needed high doses of dopamine (20 μg/kg/min) and noradrenaline (0.2-0.3 μg/kg/ min) to maintain normotension. The patient was also found to have altered coagulation parameters which were corrected with fresh frozen plasma. An emergency laparotomy revealed jejunal perforation which was closed. The patient was found to have cirrhotic liver (he was a known chronic alcoholic). Blood pressure remained persistently low, requiring addition of vasopressin infusion in the postoperative period. After multiple failed attempts at cannulation of the radial arteries in both upper limbs, a femoral arterial line was secured in the intensive care unit. By the second postoperative day, all the vasopressors were weaned off. However, cyanosis of the extremities was noted. On the third postoperative day, small blebs/blisters were noticed first in the left hand and subsequently in other limbs also which progressed to gangrene formation over the next 1 week (Figs. 1, 2, 3). Doppler evaluation revealed presence of diminished pulsations in all the vessels supplying the limbs. Echocardiogram revealed absence of any septic foci. While we were waiting for a clear demarca...

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Adult Purpura Fulminans and Digital Necrosis Associated With Sepsis and the Factor V Mutation

Cited by 15 publications

References 4 publications

Outcome in children with purpura fulminans: Report on 16 patients

Outcome in children with purpura fulminans: Report on 16 patients

Severe digital necrosis in an elderly patient with heterozygous factor V Leiden mutation

Symmetrical Peripheral Gangrene—A Case Report and Brief Review

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