A structured approach to the management of purpura fulminans.

A 5-year-old boy with purpura fulminans (PF) was successfully managed with a protocol in which fresh frozen plasma (FFP) was administered, followed by a trial of certain therapeutic agents. This approach was based upon combined experience both with the reference patient and with a subject with a chronic form of PF. FFP controlled the acute disseminated intravascular coagulation in both instances and permitted venous antithrombotic drugs to be evaluated in safety. The PF syndrome in the index case was found to be heparin responsive, while the atypical case was coumarin responsive (heparin resistant). Initial administration of FFP was recommended rather than heparin in order to minimize the risk of hemorrhage while maintaining the likelihood of a swift response. When FFP is effective, a sequential trial should be undertaken with agents from the following categories: (1) venous antithrombotic, (2) antiplatelet, (3) antifibrinolytic, and (4) antiproteolytic. This process permits therapies to be thoroughly tested and used as investigative probes into the mechanisms of a particular case of PF. Should FFP prove ineffective, the list can serve as a guide for the investigation of various fastacting agents in the acute phases of disseminated intravascular coagulation. PF treatments are ranked in accordance with the number of positive outcomes in the literature.