Diagnosis and management of neonatal purpura fulminans

Price, Victoria; Ledingham, D L; Krümpel, Anne; Chan, Anthony

doi:10.1016/j.siny.2011.07.009

Cited by 82 publications

(56 citation statements)

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“…9 Extremely low plasma levels determined by homozygous or compound heterozygous mutations in the protein S gene are rare and associated with the onset of purpura fulminans in the neonatal period. 1 Symptomatic newborns need longterm anticoagulation, and to date the recommended treatment is a vitamin K antagonist. However, despite maintaining an international normalized ratio of the prothrombin time range within the therapeutic range for the treatment venous thromboembolism, or even much higher in our child, patients with severe deficiency of protein S or protein C may develop warfarininduced skin necrosis.…”

Section: Discussionmentioning

confidence: 99%

“…Neonatal purpura fulminans is a rare, life-threatening condition caused by severe congenital deficiencies of the natural anticoagulant protein C or S. 1 The clinical presentation is that of skin necrosis and disseminated intravascular coagulation, which can progress rapidly to multiorgan failure caused by thrombotic occlusion of small-and medium-size blood vessels. Its early recognition and prompt treatment with fresh-frozen plasma, anticoagulants such as heparins or vitamin K antagonists, and, in the case of protein C deficiency, concentrate replacement therapy are essential to reduce mortality and prevent major sequelae.…”

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Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency

et al. 2013

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We report a case of a 6-year-old girl with severe protein S deficiency due to a homozygous mutation and recurrent episodes of skin necrosis. She developed purpura fulminans at birth and a catheter-related venous thrombosis complicated by massive pulmonary embolism at the sixth day of life. Long-term oral anticoagulant therapy with a vitamin K-antagonist was started with a therapeutic range of the international normalized ratio of prothrombin time between 2.0 and 3.0. Unfortunately, this common range was not sufficient because recurrent episodes of warfarin-induced skin necrosis developed if the international normalized ratio was ,4.0. Vitamin K antagonists decrease plasma level of vitamin K-dependent coagulation proteins, including the natural anticoagulant protein C. In our patient, the hypercoagulable state due to warfarin-induced reduction of protein C, other than severe protein S deficiency, outweighed the anticoagulant efficacy of the inhibition of procoagulant factors II, VII, IX, and X. The switch of anticoagulant therapy from warfarin to rivaroxaban, a direct inhibitor of activated factor X that does not inhibit other vitamin K-dependent proteins, resulted in the disappearance of skin necrosis at 1 year of follow-up. Rivaroxaban may be considered as a valid anticoagulant alternative in patients with severe inherited protein S deficiency and warfarin-induced skin necrosis.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency

et al. 2013

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“…Half of PC-deficient newborns presented cerebral sinovenous thrombosis and stroke. In this setting, PC-deficient infants characterized the phenotype of pediatric thrombophilias (purpura fulminans and intracranial lesions) beyond the ethnicity (17). The age-specific prevalence and phenotype of pediatric PC deficiency may be useful in the genetic screening for pediatric thrombophilias.…”

Section: Discussionmentioning

confidence: 99%

Age-specific onset and distribution of the natural anticoagulant deficiency in pediatric thromboembolism

et al. 2015

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“…PC deficiency in humans manifests in a variety of cutaneous signs, such as ecchymoses and rapidly progressive necrosis of the skin, as occurs in purpura fulminans [24,25,26,27]. Homozygous PC deficiency often results in neonatal purpura fulminans and is usually fatal [28]. …”

Section: Protective Effects Of Apc In Skinmentioning

confidence: 99%

Novel Functions of the Anticoagulant Activated Protein C in Maintaining Skin Barrier Integrity to Impact on Skin Disease

Xue

Jackson²

2015

Pathobiology

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The epidermis is the outermost skin layer and provides the first line of defence against the external environment. Keratinocytes are the most predominant cells in the epidermis and play a critical role in maintaining epidermal barrier function. When the barrier is disrupted any of a number of diseases, such as chronic wounds, psoriasis, pemphigus, atopic dermatitis or toxic epidermal necrolysis, can take hold. Activated protein C (APC) or its precursor, protein C, is abundantly expressed by skin epidermal keratinocytes and stimulates their proliferation and migration, and inhibits apoptosis and inflammation, leading to a healing phenotype. Importantly, APC also increases the barrier function of keratinocytes by promoting expression and cell-cell contact redistribution of tight junction proteins. These cytoprotective properties of APC on epidermal keratinocytes place it as an exciting new therapy for skin disorders associated with the disruption of barrier function and inflammation.

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Diagnosis and management of neonatal purpura fulminans

Cited by 82 publications

References 50 publications

Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency

Anticoagulant Treatment With Rivaroxaban in Severe Protein S Deficiency

Age-specific onset and distribution of the natural anticoagulant deficiency in pediatric thromboembolism

Novel Functions of the Anticoagulant Activated Protein C in Maintaining Skin Barrier Integrity to Impact on Skin Disease

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