PURPURA FULMINANS

Course overview

PURPURA FULMINANS IS AN ACUTE PURPURIC RASH CHARACTERIZED BY COAGULATION OF THE MICROVASCULATURE, WHICH LEADS TO PURPURIC LESIONS AND SKIN NECROSIS. IT IS A TRUE DERMATOLOGICAL EMERGENCY AND REQUIRES IMMEDIATE DIAGNOSIS AND MANAGEMENT. PATIENTS ARE OFTEN ACUTELY ILL WITH FEVER, HAVE HEMORRHAGE FROM MULTIPLE SITES, AND MAY BE HYPOTENSIVE. IT IS RAPIDLY PROGRESSIVE AND IS OFTEN ACCOMPANIED BY DISSEMINATED INTRAVASCULAR COAGULATION AND CIRCULATORY COLLAPSE. IT OCCURS IN NEONATES, CHILDREN, AND ADULTS. THERE ARE 3 FORMS OF THIS DISEASE WITH A CLASSIFICATION SCHEME BASED ON THE TRIGGERING MECHANISM. THE MORTALITY RATE HAS BEEN DECREASING WITH SUPPORTIVE CARE, IMPROVED MANAGEMENT OF SECONDARY COMPLICATIONS, AND SOME TARGETED TREATMENTS, BUT IT REMAINS A DISABLING CONDITION OFTEN RESULTING IN MAJOR AMPUTATIONS IN THOSE WHO SURVIVE. THIS ACTIVITY DESCRIBES THE PATHOPHYSIOLOGY AND EVALUATION OF PURPURA FULMINANS AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.