From the Guidelines
Purpura fulminans has a variable survival rate, with mortality ranging from 20% to 60% depending on several factors including the underlying cause, timing of intervention, and severity of the condition. The survival rate of Purpura Fulminans is influenced by the underlying cause, with neonatal purpura fulminans associated with protein C or S deficiency tend to have better outcomes when diagnosed and treated early 1. In contrast, infectious purpura fulminans, particularly meningococcal-associated, carries a higher mortality rate.
Factors Influencing Survival Rate
- Underlying cause of Purpura Fulminans
- Timing of intervention
- Severity of the condition
- Presence of protein C or S deficiency
- Presence of infectious causes, such as meningococcal infection
Treatment and Management
Immediate treatment is crucial and includes addressing the underlying cause (antibiotics for infectious cases), replacing deficient coagulation factors (protein C concentrate at 100 IU/kg initially, followed by 50 IU/kg every 6-12 hours), anticoagulation (typically heparin), and supportive care 1. Fresh frozen plasma (10-20 mL/kg) may be used if protein C concentrate is unavailable. Surgical debridement of necrotic tissue and amputation of gangrenous limbs are sometimes necessary.
Long-term Complications
Even with survival, patients often face significant long-term complications including skin grafting requirements, amputations, and end-organ damage 1. The high mortality rate is due to the rapid progression of disseminated intravascular coagulation, microvascular thrombosis, and subsequent multi-organ failure that characterizes this condition. The use of anticoagulation plus protein C replacement rather than anticoagulation alone in pediatric patients with congenital purpura fulminans due to homozygous protein C deficiency is recommended 1.
From the Research
Survival Rate of Purpura Fulminans
The survival rate of Purpura Fulminans is a critical aspect of understanding the severity and prognosis of this condition. According to various studies:
- The reported mortality rate of Purpura Fulminans is around 50% due to multiple organ failure 2.
- Another study suggests that the mortality rate can be as high as 60% in patients with meningococcal disease 3.
- A case report highlights the importance of early recognition and treatment, with a mortality rate remaining high despite optimal management 3.
- Replacement therapy with protein C concentrate has shown promising results, with six out of eight patients surviving in one study 4.
- The use of recombinant protein C activator has also been reported, with two out of three patients being discharged from the intensive recovery care unit 5.
Factors Affecting Survival Rate
Several factors can influence the survival rate of Purpura Fulminans, including:
- Early recognition and treatment of the underlying cause 6.
- Prompt management of disseminated intravascular coagulation and septic shock 4.
- The use of adjunctive therapies such as protein C concentrate or recombinant protein C activator 4, 5.
- The severity of organ failure and the presence of comorbidities 2, 3.