From the Guidelines
Hyperbaric oxygen therapy (HBO) is not recommended as a treatment for Purpura Fulminans, as there is no evidence to support its use in this condition. The provided evidence does not mention HBO therapy as a treatment option for Purpura Fulminans. Instead, the evidence suggests that treatment consists of long-term maintenance therapy, as well as therapy required for acute exacerbations, including anticoagulation and protein C replacement therapy 1.
Treatment Options
The current management of protein C deficiency, which is associated with Purpura Fulminans, includes oral anticoagulants, low molecular weight heparin, and intravenous or subcutaneous protein C concentrate 1. Liver transplantation (LT) is also considered a curative option, typically in the setting of multivisceral transplantation following an abdominal catastrophe due to mesenteric vein thrombosis or renal vein thrombosis 1.
Key Considerations
- The lack of evidence supporting the use of HBO therapy in Purpura Fulminans suggests that it should not be considered a treatment option.
- The focus should be on standard treatments, including anticoagulation, protein C replacement therapy, and liver transplantation, as recommended by current guidelines 1.
- Early consultation with specialists, including infectious disease specialists and hyperbaric medicine experts, is essential for optimal management of this life-threatening condition. However, in the absence of evidence supporting HBO therapy, this consultation should focus on standard treatment options.
From the Research
Role of Hyperbaric Oxygen Therapy in Purpura Fulminans
The use of hyperbaric oxygen (HBO) therapy in the treatment of Purpura Fulminans (PF) has been explored in several studies. Key points regarding its role include:
- HBO therapy has been found to be a useful adjunct in the management of PF, particularly in reducing ischemic tissue and demarcating blackened tissue 2.
- The effectiveness of HBO therapy is associated with the timeliness and aggressiveness of its initiation, with most cases showing improvement when treated promptly 2.
- HBO therapy has been used in various cases of PF, including those caused by sepsis, congenital or acquired protein C or protein S deficiency, and other underlying conditions 2, 3.
- The therapy is believed to impose minimal side effects or complications, making it a viable option for the management of PF complications 2.
Mechanism and Management of Purpura Fulminans
The mechanism of PF involves a failure of the anticoagulant protein C pathway, leading to uncontrolled microvascular clotting and inadequate protein C-mediated cytoprotective effects 4, 5.
- Management of PF involves treatment of the underlying infection, aggressive anticoagulation, and robust transfusion support aimed at correcting acquired deficiencies in natural anticoagulant proteins 4, 5.
- Hyperbaric oxygen therapy has been used as a curative treatment in some cases, particularly in extremely low birth weight preterm infants 3.
Forms and Classification of Purpura Fulminans
PF can be classified into three forms: neonatal purpura fulminans, idiopathic purpura fulminans, and acute infectious purpura fulminans 6.
- Each form has distinct triggering mechanisms and underlying causes, including hereditary deficiencies of natural anticoagulants and acquired deficiencies due to infection or other factors 6.