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What are purpura fulminans?

What are purpura fulminans?

Purpura fulminans (PF) is a haematological emergency in which there is skin necrosis and disseminated intravascular coagulation. This may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels.

How do you treat purpura fulminans?

Management of acute infectious purpura fulminans may include the following: Empiric, broad-spectrum intravenous antibiotic therapy against Neisseria meningitidis, streptococci, and methicillin-resistant Staphylococcus aureus (MRSA) Early administration of APC concentrates. Intravenous immunoglobulin (IVIg) therapy.

What does purpura fulminans look like?

The initial appearance of purpura fulminans lesions is of well-demarcated erythematous lesions which progress rapidly to develop irregular central areas of blue-black haemorrhagic necrosis. Advancing areas of necrosis are often surrounded by a thin border of erythema that fades into adjacent unaffected skin.

What is neonatal purpura fulminans?

Abstract. Neonatal purpura fulminans is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. The condition is often fatal unless there is early recognition of the clinical symptoms, prompt diagnosis, and judicious replacement therapy is initiated.

What is Purpura sepsis?

Sepsis-associated Purpura fulminans (SAPF) is a rare life-threatening condition. It is characterized by multiple skin lesions which rapidly progress to necrosis and gangrene. SAPF is a manifestation of widespread clot formation in small blood vessels which emerges secondarily to severe bacterial and viral infections.

What causes purpura in sepsis?

Protein C/Activated Protein C Dysfunction of the protein C pathway during sepsis is one of the major factors contributing to the development of purpura fulminans. Severe depletion of protein C leads to uncontrolled hemorrhage and thrombosis and is associated with poor outcome.

How is purpura fulminans diagnosed?

Specific levels of antithrombin III, free protein C, and free and total protein S may help confirm the diagnosis especially in the neonatal form of the disease. Otherwise, the evaluation of patients with purpura fulminans mimics the evaluation of the underlying cause.

How long do purpura spots last?

Most purpuric lesions last between one and three weeks, though the discoloration may be permanent after they fade. You can talk to your dermatologist about how to reduce their appearance.

Should I worry about purpura?

Patients who experience purpura with any of the following symptoms should seek medical treatment: Low platelet count, which may lead to increased bleeding after an injury, bleeding gums or nose, or blood in urine or bowel movements. Sore, swollen joints, particularly in the ankles and knees.

What does purpura fulminans mean in medical terms?

Purpura fulminans. Jump to navigation Jump to search. Purpura fulminans is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising and discolouration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leads to skin necrosis and disseminated intravascular coagulation.

How long does it take for purpura fulminans to heal?

Purpura fulminans lesions, once established, often progress within 24 to 48 hours to full-thickness skin necrosis or soft-tissue necrosis. Once purpura fulminans lesions progress to full-thickness skin necrosis, healing takes between 4–8 weeks and leaves large scars.

What causes purpura fulminans after chicken pox?

In some cases, a combination of sepsis and a partial congenital defect in the protein C anticoagulant pathway initiates purpura fulminans. In rare instances, purpura fulminans is an autoimmune manifestation against protein C or protein S after normally benign infections, such as chicken pox. Sometimes purpura fulminans has unknown cause.

How to treat purpura fulminans with protein C concentrate?

Purpura fulminans with disseminated intravascular coagulation should be urgently treated with fresh frozen plasma (10–20 mL/kg every 8–12 hours) and/or protein C concentrate to replace pro-coagulant and anticoagulant plasma proteins that have been depleted by the disseminated intravascular coagulation process.