title: Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia--A Tertiary Center's Experience.
abstract: Purpura fulminans (PF) is a very rare clinicopathologic skin disorder comprising dermal microvascular thrombosis associated with perivascular hemorrhage of multiple origins. It may occur as the presenting symptom of severe congenital deficiency of protein C (PC) or protein S (PS) during the newborn period, or later in life following oral anticoagulant therapy with vitamin K antagonists, or of sepsis that may be associated with disseminated intravascular coagulation. Treatment consists of anticoagulants and PC concentrates during acute episodes. We report our experience in the diagnosis and management of pediatric PF. The medical records of the 6 children aged 2-16 years (median: 5 years) who presented with PF to our tertiary care center between 1996 and 2013 were studied. The thrombophilia workup revealed either the presence of congenital homozygous PC deficiency, prothrombotic polymorphisms (factor V Leiden and FIIG20210A heterozygosity), acquired PC/PS deficiency, or no discernible thrombophilia. The skin necrosis resolved following conservative fresh-frozen plasma/anticoagulant therapy in 2 cases, whereas 3 children required interventional plastic surgery. The sixth case, a 10-year-old child with severe PC deficiency, heterozygous factor V Leiden, and FIIG20210A, received recombinant activated PC. PF in childhood is rare and has multiple etiologies. Understanding of the variable pathogenesis and risk factors will facilitate diagnosis and appropriate clinical management.
authors.affiliation: a Department of Pediatric Emergency Care and Pediatric Hematology Unit , Soroka Medical Center , Beer-Sheba , Israel , affiliated to the Ben Gurion University of the Negev , Beer Sheva , Israel.|b Thrombosis Unit, National Hemophilia Center , Safra Children's Hospital, Sheba Medical Center , Tel Hashomer, Israel.
authors.forename: Yariv
authors.initials: Y
authors.lastname: Fruchtman
authors.affiliation: b Thrombosis Unit, National Hemophilia Center , Safra Children's Hospital, Sheba Medical Center , Tel Hashomer, Israel.|c Department of Neonatology , Safra Children's Hospital, Sheba Medical Center , Tel Hashomer, Israel.
authors.forename: Tzipora
authors.initials: T
authors.lastname: Strauss
authors.affiliation: d Department of Critical Care, Safra Children's Hospital , Sheba Medical Center , Tel Hashomer , Israel , affiliated to the Sackler Faculty of Medicine , Tel Aviv University , Tel Aviv , Israel.
authors.forename: Marina
authors.initials: M
authors.lastname: Rubinstein
authors.affiliation: a Department of Pediatric Emergency Care and Pediatric Hematology Unit , Soroka Medical Center , Beer-Sheba , Israel , affiliated to the Ben Gurion University of the Negev , Beer Sheva , Israel.
authors.forename: Miriam
authors.initials: M
authors.lastname: Ben Harush
authors.affiliation: e Department of Pediatric Hematology/Oncology , Hadassah Hebrew University Medical Center , Jerusalem , Jerusalem , Israel.
authors.forename: Shoshana
authors.initials: S
authors.lastname: Revel-Vilk
authors.affiliation: a Department of Pediatric Emergency Care and Pediatric Hematology Unit , Soroka Medical Center , Beer-Sheba , Israel , affiliated to the Ben Gurion University of the Negev , Beer Sheva , Israel.
authors.forename: Joseph
authors.initials: J
authors.lastname: Kapelushmik
authors.affiliation: d Department of Critical Care, Safra Children's Hospital , Sheba Medical Center , Tel Hashomer , Israel , affiliated to the Sackler Faculty of Medicine , Tel Aviv University , Tel Aviv , Israel.
authors.forename: Gideon
authors.initials: G
authors.lastname: Paret
authors.affiliation: b Thrombosis Unit, National Hemophilia Center , Safra Children's Hospital, Sheba Medical Center , Tel Hashomer, Israel.
authors.forename: Gili
authors.initials: G
authors.lastname: Kenet
mesh_terms: D002648:Child; D002675:Child, Preschool; D005165:Factor V / Q000235:genetics*; D005260:Female; D006801:Humans; D008297:Male; D010949:Plasma; D011110:Polymorphism, Genetic; D055665:Purpura Fulminans / Q000150:complications / Q000188:drug therapy / Q000235:genetics; D012189:Retrospective Studies; D012871:Skin Diseases / Q000150:complications / Q000188:drug therapy / Q000235:genetics; D062606:Tertiary Care Centers; D019851:Thrombophilia / Q000150:complications / Q000188:drug therapy / Q000235:genetics
chemical_list: C095381:factor V Leiden; D005165:Factor V
keywords: anticoagulant; factor V Leiden; protein C; protein S; skin necrosis; thrombosis