Postinfectious purpura fulminans: A case report
Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of c...
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Veröffentlicht in: | Archivos argentinos de pediatría 2024-08, Vol.122 (4), p.e202310137-e202310137 |
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description | Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibody-mediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months. |
doi_str_mv | 10.5546/aap.2023-10137.eng |
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It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibody-mediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. 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source | MEDLINE; DOAJ Directory of Open Access Journals; EZB Electronic Journals Library |
subjects | Child Disseminated Intravascular Coagulation - diagnosis Disseminated Intravascular Coagulation - etiology Humans Male Protein S Deficiency - complications Protein S Deficiency - diagnosis Purpura Fulminans - diagnosis Purpura Fulminans - etiology |
title | Postinfectious purpura fulminans: A case report |
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