Acute Infectious Purpura Fulminans: A 15-Year Retrospective Review of 28 Consecutive Cases

Abstract

Acute infectious purpura fulminans (AIPF) is a rare syndrome of hemorrhagic infarction of the skin, extremity loss, and intravascular thrombosis. It progresses rapidly and is accompanied by disseminated intravascular coagulation and vascular collapse. The victims often succumb to the disease. Our objective was to investigate the clinical manifestations, outline the clinical course, and delineate factors related to mortality among the patients with AIPF. Patients diagnosed with AIPF over a 15-year period were reviewed retrospectively for patient history, comorbid conditions, progression of clinical course, and medical and surgical management. The vast majority of the patients were under the age of 7; however, the disease process can be seen in adults. The overall mortality rate was 43 per cent. The major predisposing factors were history of recent upper respiratory infection, recent surgery or childbirth, young age, and absence of a spleen. The most common clinical manifestations were skin discoloration, disseminated intravascular coagulation, fever, and septic shock. The most common bacteria cultures were Neisseria meningitidis, Hemophilus influenzae, and Streptococcus pneumoniae. There appears to be a higher mortality in patients who did not undergo a surgical intervention. Compartment syndrome needs to be evaluated early on in the presentation. Rapid diagnosis, intensive care unit management, and prompt surgical consultation and debridement may decrease the mortality.