From the Guidelines
Purpura fulminans is a life-threatening condition that can lead to severe outcomes, including extensive skin necrosis, disseminated intravascular coagulation (DIC), multiorgan failure, and death, with a mortality rate of 20-60% despite modern intensive care. The primary outcomes of purpura fulminans include:
- Extensive skin necrosis
- Disseminated intravascular coagulation (DIC)
- Multiorgan failure
- Death Survivors often face significant long-term consequences, such as:
- Amputation of affected limbs
- Skin grafting requirements
- Permanent scarring
- Neurological complications, including seizures, cognitive impairment, and peripheral neuropathy
- Renal failure, sometimes requiring dialysis or even transplantation in severe cases
- Adrenal hemorrhage, leading to adrenal insufficiency, necessitating lifelong hormone replacement therapy, as noted in studies such as 1 and 1.
Early aggressive treatment with antibiotics, anticoagulation therapy, blood product replacement, protein C concentrate for protein C deficiency cases, and surgical debridement of necrotic tissue is essential to improve outcomes, as suggested by the American Society of Hematology 2018 guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism 1. The use of protein C replacement rather than anticoagulation in pediatric patients with congenital purpura fulminans due to homozygous protein C deficiency is recommended, as it has been shown to have superior long-term effectiveness and reduced adverse bleeding risk 1. However, the optimal therapy depends on the values and preferences of the family, as well as local health service factors, and liver transplantation may be considered as a curative option for protein C deficiency, as noted in 1.
From the Research
Outcomes of Purpura Fulminans
The outcomes of Purpura Fulminans (PF) can be severe and life-threatening. Some of the possible outcomes include:
- High mortality rate, with a reported mortality rate as high as 60% in patients with meningococcal disease 2
- Multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels 3
- Amputation of involved extremities and significant scarring in affected areas 4, 5
- Irreversible tissue necrosis within 48 hours 2
- Disseminated intravascular coagulation, thrombotic complications, and purpura fulminans in severe congenital protein C deficiency 6
Long-term Health Sequelae
Long-term health sequelae can be significant, including:
- Major long-term health sequelae if not recognized and treated early 3
- Morbidity and mortality remain high, even with prompt recognition and treatment 2
- Need for long-term management and anticoagulation therapy to prevent further thrombotic complications 6
Management and Treatment
Management and treatment of PF require a multidisciplinary approach, including: