Severe acquired protein C deficiency in purpura fulminans associated with disseminated intravascular coagulation: treatment with protein C concentrate

Purpura fulminans (PF) defines an acute, often lethal syndrome of disseminated intravascular coagulation (DIC) with rapidly progressive hemorrhagic necrosis of the skin due to dermal vascular thrombosis.1-7 It is indicative of a severe disturbance in hemostasis now recognized to involve the protein...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1993-02, Vol.91 (2), p.418-422
Main Authors: Gerson, W T, Dickerman, J D, Bovill, E G, Golden, E
Format: Article
Language:English
Subjects:
Adolescent
Bacteremia - complications
Blood
Blood Coagulation Factors - analysis
Blood Coagulation Factors - drug effects
Care and treatment
Case studies
Chickenpox - complications
Disseminated intravascular coagulation
Disseminated Intravascular Coagulation - blood
Disseminated Intravascular Coagulation - etiology
Humans
Infusions, Intravenous
Injections, Intravenous
Male
Medical disorders
Medical research
Pediatrics
Protein C - administration & dosage
Protein C - therapeutic use
Protein C Deficiency
Proteins
Purpura
Purpura (Pathology)
Purpura - drug therapy
Purpura - etiology
Shock, Septic - complications
Streptococcal Infections - complications
Streptococcus pyogenes
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Summary:Purpura fulminans (PF) defines an acute, often lethal syndrome of disseminated intravascular coagulation (DIC) with rapidly progressive hemorrhagic necrosis of the skin due to dermal vascular thrombosis.1-7 It is indicative of a severe disturbance in hemostasis now recognized to involve the protein C system in many cases.1,2,5,8-12 Purpura fulminans is usually seen in three clinical settings: (1) in the newborn period as a manifestation of homozygous protein C deficiency, or rarely protein S deficiency13,14; (2) in individuals with acute, severe viral or bacterial infection where an acquired deficiency in protein C activity is documented1-3,5-8,10,12,15; and (3) as a rare, postinfectious syndrome with a history of an antecedent" preparatory disease," most commonly a viral or bacterial illness involving the skin (eg, varicella or scarlet fever), with the sudden development, during an otherwise unremarkable convalescence, of progressive purpura and necrosis.3-5,7,10,12
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.91.2.418