title: [Idiopathic purpura fulminans with transient protein S deficiency].
abstract: Idiopathic purpura fulminans produces rapidly progressive hemorrhagic necrosis of the skin with disseminated intravascular coagulation in individuals without known abnormalities of the protein C pathway or acute infections. The disease mainly affects children and in 90 % of cases is preceded by a benign infection. Its pathogenesis involves a temporary autoimmune protein S deficiency that provokes a state of hypercoagulability. We present the case of a previously healthy 2-year-old boy with hemorrhagic skin lesions characteristic of purpura fulminans and disseminated intravascular coagulation without sepsis. Severe, temporary protein S deficiency was confirmed. The patient received daily replacement therapy with fresh frozen plasma for 12 days and anticoagulation with heparin for 3 months. Evolution was favorable. Although the other parameters returned to normal, protein S remained low for 50 days despite treatment. The patient has made a complete recovery.
authors.affiliation: Unidad de Cuidados Intensivos Pediátricos.Hospital Donostia. San Sebastián. ja1111@euskalnet.net
authors.forename: C
authors.initials: C
authors.lastname: de Frutos Martínez
authors.forename: A
authors.initials: A
authors.lastname: Iturrioz Mata
authors.forename: E
authors.initials: E
authors.lastname: González Pérez-Yarza
authors.forename: M C
authors.initials: MC
authors.lastname: Arratibel Fuentes
authors.forename: R
authors.initials: R
authors.lastname: Sainz Arroniz
authors.forename: Y
authors.initials: Y
authors.lastname: Albisu Andrade
mesh_terms: D002675:Child, Preschool; D006801:Humans; D011695:IgA Vasculitis / Q000209:etiology*; D008297:Male; D018455:Protein S Deficiency / Q000150:complications*; D012720:Severity of Illness Index