From the Guidelines
Purpura fulminans neuropathy requires immediate aggressive management with anticoagulation therapy and addressing the underlying cause, such as protein C deficiency, to prevent irreversible neurological damage. The condition is a rare but serious neurological complication of purpura fulminans, characterized by rapidly progressive skin necrosis and disseminated intravascular coagulation. The neuropathy develops due to microvascular thrombosis affecting the peripheral nerves, leading to ischemia and subsequent nerve damage 1.
Treatment Approach
- Anticoagulation therapy with unfractionated heparin initially, followed by low molecular weight heparin, is crucial in managing purpura fulminans neuropathy.
- For cases related to protein C deficiency, protein C concentrate (50-100 IU/kg initially, followed by 50 IU/kg every 6-12 hours) should be administered 1.
- Supportive care for the neuropathy includes pain management with gabapentin (300-1200 mg daily in divided doses) or pregabalin (150-600 mg daily in divided doses), physical therapy to prevent contractures, and rehabilitation.
- Early recognition and intervention are crucial to prevent irreversible neurological deficits, as the neurological damage may be permanent in some cases, depending on the extent of ischemic injury to the nerves and how quickly the underlying condition was treated.
Recent Findings
Recent studies have highlighted the importance of prompt treatment in managing purpura fulminans neuropathy, although they may not directly address the condition, they emphasize the need for aggressive management of related conditions 1. The focus should always be on preventing morbidity, mortality, and improving the quality of life for patients with this severe condition.
From the Research
Purpura Fulminans Neuropathy
There is limited information available on purpura fulminans neuropathy. However, based on the available studies, here are some key points:
- Purpura fulminans (PF) is a haemorrhagic condition usually associated with sepsis or previous infection, characterized by tissue necrosis, small vessel thrombosis, and disseminated intravascular coagulation 2.
- The condition can lead to severe scarring and tissue loss, and in some cases, amputation of involved extremities 2, 3.
- 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.
- Management of PF involves treatment of the underlying infection, aggressive anticoagulation, and robust transfusion support aimed at correcting acquired deficiencies in natural anticoagulant proteins 3, 5.
- In cases of severe genetic protein C deficiency, recombinant activated protein C (APC) has been used to treat PF, with positive results 6.
Key Features of Purpura Fulminans
- Highly thrombotic subtype of disseminated intravascular coagulation
- Associated with severe bacterial or viral infections
- Characterized by tissue necrosis, small vessel thrombosis, and disseminated intravascular coagulation
- High mortality rate, with survivors often experiencing amputation of involved extremities and significant scarring
Management Strategies
- Treatment of the underlying infection
- Aggressive anticoagulation
- Robust transfusion support aimed at correcting acquired deficiencies in natural anticoagulant proteins
- Use of recombinant activated protein C (APC) in cases of severe genetic protein C deficiency 6