Presentation and outcome of purpura fulminans associated with peripheral gangrene in 12 patients at Mayo Clinic

BackgroundDermatologists may be called to assist in the diagnosis and management of purpura fulminans.MethodsThis retrospective case series details the clinical presentation and outcomes of patients presenting with purpura fulminans associated with peripheral gangrene between 1989 and 2004.ResultsAll 12 patients presented with sudden onset of purpuric patches and evolving gangrene of the extremities in association with a shock syndrome (hypotension, oliguria). Eleven patients had disseminated intravascular coagulation. The cause of purpura fulminans was infectious in 9 patients, surgical in two, and cancer in one. Three patients died (25%) within a week of onset of purpura fulminans. Of the 9 surviving patients, 8 required amputation of at least one limb. Four patients required amputation of all 4 limbs.LimitationsRetrospective study design, varying clinical descriptions, and potential referral bias are limitations.ConclusionPurpura fulminans in association with symmetric peripheral gangrene is an ominous clinical presentation.