[Idiopathic purpura fulminans with transient protein S deficiency].
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.
de Frutos Martínez
C
C
Unidad de Cuidados Intensivos Pediátricos.Hospital Donostia. San Sebastián. ja1111@euskalnet.net
Iturrioz Mata
A
A
González Pérez-Yarza
E
E
Arratibel Fuentes
M C
MC
Sainz Arroniz
R
R
Albisu Andrade
Y
Y
D002675:Child, Preschool; D006801:Humans; D011695:IgA Vasculitis / Q000209:etiology*; D008297:Male; D018455:Protein S Deficiency / Q000150:complications*; D012720:Severity of Illness Index