Purpura fulminans in adult patients

Abstract

Purpura fulminans is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpuric rash together with an acute circulatory failure. PF commonly affects young patients with no previous comorbidities. Neisseria meningitidis and Streptococcus pneumoniae are the leading causative bacteria. Diagnosing purpura fulminans before the apparition of the purpuric rash is challenging since prodromal symptoms are nonspecific and consistent with a “flu-like” syndrome. The clinical presentation of patients with purpura fulminans differs from that of patients with bacterial meningitis since most of the patients with purpura fulminans have no neurological impairment. Microbiological diagnosis relies on blood cultures and skin biopsy of purpuric lesions which is a non-invasive examination allowing to detect the causative bacteria up to 72 hours after antibiotics initiation. The indication for lumbar puncture must be evaluated on a case-by-case basis because patients usually have no neurological signs but severe coagulation disorders. Treatment is no different from that of any other septic shock: antibiotic therapy with a third-generation cephalosporin as soon as the diagnosis is suspected and treatment of organ failure with particular attention to myocardial and adrenal involvement. Despite these pathogens being highly susceptible to broadly available antibiotics, the prognosis of PF is dismal with a mortality rate of 41% in the intensive care unit and a significant risk of distant sequelae in surviving patients.