From the Guidelines
Purpura fulminans is not considered a type of hypersensitivity reaction, but rather a life-threatening condition characterized by disseminated intravascular coagulation and skin necrosis. The management of purpura fulminans requires immediate, aggressive intervention focused on treating the underlying cause, supporting organ function, and preventing complications. Treatment begins with prompt administration of broad-spectrum antibiotics such as vancomycin (15-20 mg/kg IV every 8-12 hours) plus a carbapenem like meropenem (1g IV every 8 hours) or ceftriaxone (2g IV daily) if meningococcal or other bacterial infection is suspected 1. Simultaneously, patients need hemodynamic support with IV fluids (crystalloids at 20-30 ml/kg bolus initially) and vasopressors like norepinephrine (starting at 0.05-0.1 mcg/kg/min) for persistent hypotension. Replacement of coagulation factors is critical, using fresh frozen plasma (15-20 ml/kg), cryoprecipitate (1 unit per 5-10 kg body weight), and platelet transfusions (1 unit per 10 kg) to correct disseminated intravascular coagulation. Protein C concentrate (50-100 IU/kg initially, then 50 IU/kg every 6-12 hours) should be administered if available, particularly in cases of congenital protein C deficiency 1. Heparin therapy (unfractionated heparin at 10-15 units/kg/hour) may be considered to prevent further thrombosis. Surgical consultation is essential for wound care and possible debridement of necrotic tissue, while skin grafting may be needed later for extensive tissue damage. Supportive care includes mechanical ventilation, renal replacement therapy, and nutritional support as needed. Early involvement of a multidisciplinary team including critical care, hematology, infectious disease, and surgical specialists is crucial for optimal management of this rapidly progressive, life-threatening condition. In cases of congenital purpura fulminans due to homozygous protein C deficiency, liver transplantation may be considered as a curative option, although it has its own risks and burden of care 1.
From the Research
Definition and Classification of Purpura Fulminans
- Purpura fulminans (PF) is a haematological emergency characterized by skin necrosis and disseminated intravascular coagulation, which may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels 2.
- It is not explicitly classified as a type of hypersensitivity reaction in the provided studies, but rather as a highly thrombotic subtype of disseminated intravascular coagulation that can accompany severe bacterial or viral infections 3.
Management and Treatment of Purpura Fulminans
- Early recognition and treatment of PF is essential to reduce mortality and to prevent major long-term health sequelae 2.
- Management strategies require accurate identification of the underlying cause, and may include treatment of the underlying infection, aggressive anticoagulation, and robust transfusion support aimed at correcting acquired deficiencies in natural anticoagulant proteins 3.
- Repletion of natural circulating anticoagulants, use of therapeutic anticoagulation, and optimization of transfusion support are also recommended treatment approaches 4.
- A multidisciplinary approach is needed in the treatment of this often catastrophic disease, and may involve consultant hematologists to assist with diagnosis and management of patients 5.
Clinical Features and Diagnosis of Purpura Fulminans
- Patients with PF often present with widespread purpura, fever, hypotension, and multi-organ dysfunction, including renal, hepatic, and respiratory failure 6.
- Laboratory findings may reveal severe coagulopathy, with a low platelet count, higher levels of D-dimer, prolonged prothrombin time, and activated partial thromboplastin time 6.
- The fundamental pathologic lesion in PF is a failure of the anticoagulant protein C pathway, which leads to uncontrolled microvascular clotting and inadequate protein C-mediated cytoprotective effects 4.