ABSTRACT
Purpura fulminans is a rare life-threatening dermatological emergency characterized by progressive hemorrhagic infarction, tissue necrosis, and intravascular thrombosis typically involving small dermal vessels, secondary to deficiency of natural anticoagulants like protein C, S, and antithrombin. It predominantly occurs in three clinical settings: inherited coagulation defects such as homozygous protein C deficiency, postinfectious, and idiopathic. Acquired forms secondary to fulminant bacterial infections are much more common than inherited deficiencies of natural anticoagulants, which may present as neonatal purpura fulminans. Development of purpura fulminans in a setting of sepsis portends a poor prognosis for the patient. Key to successful management is early clinical suspicion, prompt replacement of deficient proteins by fresh frozen plasma, and management of associated sepsis and disseminated intravascular coagulation. Inherited forms may require lifelong anticoagulation and replacement therapy for the deficient protein.
