Abstract
In this report, we present an adult case of severe meningococcal sepsis-associated purpura fulminans (MSPF), which is rare in Japan. A 50-year-old female presented with sudden-onset high fever, sore throat, and bilateral gonalgia; subsequently, she developed disseminated intravascular coagulation (DIC), widespread purpura fulminans (PF), severe shock, and respiratory and renal failures. Blood culture findings confirmed sepsis due to Neisseria meningitidis. The initial disease condition was successfully treated with conventional supportive therapies for DIC and septic shock; however, amputation of bilateral lower extremities and all fingers was performed due to extensive soft tissue necrosis that occurred during the clinical course. With improved critical care, the mortality related to MSPF has decreased dramatically, and many unconventional treatment strategies for MSPF have been proposed recently. Nevertheless, whether these strategies have a significant impact on the outcome of MSPF remains unverified. Here, we discuss the therapies for sepsis-associated purpura fulminans along with a review of the relevant literature. Currently, the application of appropriate conventional therapies for sepsis and DIC at an early stage of the disease seems to be the optimal method of improving outcome.
