Background: Purpura Fulminans (PF) is a rare clinical-pathologic skin disorder, comprised of dermal micro-vascular thrombosis associated with perivascular hemorrhage. It may occur either in the newborn period, as the presenting symptom of severe congenital Protein C (PC) or Protein S (PS) Deficiency, or later in life, following oral anticoagulant therapy with vitamin K antagonists, or sepsis that may be associated with DIC. Patients are treated with anticoagulants and protein C concentrates during acute episodes.
Methods: We report the experience of our tertiary center, treating 5 children that presented within the last decade with PF.
Results: Thrombophilia workup yielded variable results including presence of congenital homozygous PC deficiency, pro-thrombotic polymorphisms (Factor V Leiden and FIIG20210A heterozygosity), acquired PC/PS deficiency or no definite thrombophilia. Skin necrosis resolved following conservative fresh frozen plasma (FFP)/anticoagulant therapy in 2 of the 5 cases, whereas 3 children required interventional plastic surgery. Use of recombinant activated protein C (APC) was reported in one case of a 10 year old child with severe protein C deficiency, heterozygous factor V Leiden and FIIG20210A.
Discussion: PF in childhood is a rare disease, with various Etiologies. Understanding of the pathogenesis, risk factors and available therapy, may improve the outcome of those patients.
