Isolated Cephalohematoma as Presentation of Idiophatic Purpura Fulminans

Abstract

Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic coagulation disorder. Acquired IPF is usually due to a cross-reactivity against virus and anticoagulant proteins; Protein C and less commonly Protein S. This molecular mimicry generates antibodies against anticoagulant proteins. Most used treatments are fresh frozen plasma (FFP) and heparin. In refractory cases immunoglobulins, corticosteroids and plasmapheresis can be used. Furthermore, debridement of necrotic areas is sometimes needed. Skin involvement commonly appears in lower limbs or torso, in contrast of other types of purpura fulminans were a more acral involvement is seen. Our case describes for the first time an IPF with facial location.