Modern concepts of the diagnosis and treatment of purpura fulminans

Abstract

Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The purpose of this collective review is to provide modern concepts on the diagnosis and treatment of neonatal purpura fulminans, idiopathic purpura fulminans, and acute infectious purpura fulminans. There are three forms of this disease that are classified by the triggering mechanisms. First, neonatal purpura fulminans is associated with a hereditary deficiency of the natural anticoagulants Protein C and Protein S as well as Antithrombin III. Idiopathic purpura fulminans usually follows an initiating febrile illness that manifests with rapidly progressive purpura. Deficiency of Protein S is considered to be central to the pathogenesis of this form of the disease. The third and most common type of purpura fulminans is acute infectious purpura fulminans. The mortality rate has decreased with better treatment of secondary infections, supportive care, and new treatments, but it remains a disabling condition often requiring major amputations.