Purpura Fulminans Emergency Management

Algorithm Steps

1. ▶

   Start

   Suspected Purpura Fulminans

   Acute purpuric rash with progression to skin necrosis

   1. ⚠

      Warning

      LIFE-THREATENING EMERGENCY

      Mortality 40-50%, requires immediate intervention

      • Microvascular thrombosis → tissue necrosis
      • Associated with DIC
      • Progression from erythema → purpura → necrosis
      • Multi-organ failure common
      • ICU admission mandatory
      1. ●

         Action

         Clinical Presentation

         Characteristic progression

         • Initial: Erythematous macules
         • Evolution: Blue-black hemorrhagic purpura
         • Progression: Vesicles, bullae, hard eschars
         • Pain initially, then sensory loss
         • Fever, hypotension, hemorrhage elsewhere
         • End-organ damage (renal, pulmonary)
         1. ◆

            Decision

            Classify Type of Purpura Fulminans

            Three distinct forms with different triggers

            1. ●

               Action

               Neonatal Form

               Hereditary protein C/S deficiency

               • Onset: Within 5 days of birth
               • Incidence: 1 per million live births
               • Cause: Severe protein C, S, or antithrombin III deficiency
               • Systemic thrombosis
               • Screen family members
               1. ●

                  Action

                  Immediate Resuscitation

                  ABC stabilization and sepsis management

                  • IV access, aggressive fluid resuscitation
                  • Vasopressors if needed
                  • Broad-spectrum antibiotics STAT
                  • Blood cultures before antibiotics if possible
                  • Airway management as needed
                  1. ●

                     Action

                     Antibiotic Therapy (Infectious Form)

                     Empiric coverage for likely pathogens

                     • Carbapenem (meropenem) + Vancomycin
                     • OR Ceftriaxone + Vancomycin
                     • ADD Clindamycin for toxin inhibition
                     • Adjust based on cultures
                     • Duration: Complete course for source
                     1. ●

                        Action

                        Laboratory Monitoring

                        Serial coagulation and organ function

                        • PT/PTT, fibrinogen, D-dimer (DIC panel)
                        • Platelet count
                        • Protein C, Protein S, Antithrombin III levels
                        • Lactate, organ function panels
                        • Blood cultures, wound cultures
                        • CBC with differential
                        1. ●

                           Action

                           Additional Therapies

                           Consider based on severity and response

                           • IVIG 1g/kg/day x 2 days (immunomodulation)
                           • Activated Protein C (drotrecogin alfa) - if available
                           • Corticosteroids for adrenal insufficiency
                           • Plasmapheresis (post-infectious form)
                           • Hyperbaric oxygen (limited evidence)
                           1. ⚠

                              Warning

                              Surgical Management

                              Often required for survival

                              • Early surgical consultation
                              • Fasciotomy for compartment syndrome
                              • Debridement of necrotic tissue
                              • May require amputation of extremities
                              • Skin grafting in recovery phase
                              • Timing based on patient stability
                              1. ✓

                                 Outcome

                                 Survival

                                 Significant morbidity expected

                                 • Major amputations common
                                 • Skin grafting required
                                 • Long-term rehabilitation
                                 • Genetic counseling (hereditary forms)
                                 • Lifelong anticoagulation may be needed
                              2. ⚠

                                 Warning

                                 Death

                                 Mortality 40-50%

                                 • Multi-organ failure
                                 • Refractory septic shock
                                 • Hemorrhage
                                 • Early palliative care discussion appropriate
                  2. ●

                     Action

                     DIC and Coagulation Management

                     Restore coagulation balance

                     • Fresh frozen plasma (FFP) for clotting factors
                     • Platelet transfusion if <50,000 or bleeding
                     • Cryoprecipitate if fibrinogen <100 mg/dL
                     • Protein C concentrate if available (neonatal form)
                     • Antithrombin III replacement if deficient
                     • Heparin controversial - consider if thrombosis dominant
            2. ●

               Action

               Idiopathic (Post-Infectious)

               Acquired protein S deficiency

               • Onset: 7-10 days after infection
               • Preceding: Varicella, scarlet fever most common
               • Mechanism: Anti-protein S antibodies develop
               • Transient deficiency
               • Rarest form
            3. ⚠

               Warning

               Acute Infectious Form

               Most common - occurs during acute sepsis

               • 10-20% of meningococcal septicemia
               • Pathogens: N. meningitidis, S. pneumoniae, GAS
               • Endotoxin → DIC → protein C consumption
               • Higher risk in asplenia
               • Most severe form