Purpura Fulminans

Epidemiology

Antithrombin III Deficiency (see Antithrombin III Deficiency)
Disseminated Intravascular Coagulation (DIC) (see Disseminated Intravascular Coagulation)
Protein C Deficiency (see Protein C Deficiency)
Protein S Deficiency (see Protein S Deficiency)

Capnocytophaga Canimorsus (see Capnocytophaga Canimorsus)
- Epidemiologic Exposure: dog bite
- Risk Factor for Bacteremia/Sepsis: asplenia (see Asplenia)
Cryptococcosis (see Cryptococcosis)
Escherichia Coli (see Escherichia Coli)
Enterobacter (see Enterobacter)
Haemophilus Aegyptius (see Haemophilus Aegyptius)
Haemophilus Influenzae (see Haemophilus Influenzae)
Klebsiella Pneumoniae (see Klebsiella Pneumoniae)
Neisseria Meningitidis (see Neisseria Meningitidis): common etiology
Moraxella Catarrhalis (see Moraxella Catarrhalis)
Proteus Mirabilis (see Proteus Mirabilis)
Rocky Mountain Spotted Fever (see Rocky Mountain Spotted Fever)
Staphylococcus Aureus (see Staphylococcus Aureus): recently identified etiology [MEDLINE]
Streptococcus Agalactiae (Group B Strep) (see Streptococcus Agalactiae)
Streptococcus Pneumoniae (Pneumococcus) (see Streptococcus Pneumoniae)
Streptococcus Pyogenes (Group A Strep) (see Streptococcus Pyogenes)
Varicella (see Varicella-Zoster Virus): common etiology

Acute Gastroenteritis (see Acute Gastroenteritis)
Measles (see Measles Virus)
Neisseria Meningitidis (see Neisseria Meningitidis): common etiology (most common in infants and adolescents)
Rubella (German Measles) (see Rubella Virus)
Scarlet Fever (se Scarlet Fever)
Streptococcal Pharyngitis (see Streptococcus): Streptococcus species are the most common etiology in adults
Upper Respiratory Infection
Varicella (see Varicella-Zoster Virus): common etiology

Epidemiology: usually associated with protein C, protein S, or anti-thrombin III deficiency
Diagnosis: decreased protein C, protein S, or anti-thrombin III
Clinical: occurs within the first 72 hrs after birth

Epidemiology: most common type
Physiology: involves decreased protein S resulting in severe disseminated intravascular coagulation (DIC) (see Disseminated Intravascular Coagulation)
Clinical
- Large Purpuric Skin Lesions (see Purpura)
- Fever (see Fever)
- Hypotension/Sepsis (see Sepsis)
- Disseminated Intravascular Coagulation (DIC) (see Disseminated Intravascular Coagulation)

Epidemiology
- Most cases occur in children
- Over 90% of cases are preceded by infection
Physiology: bacterial endotoxin -> imbalance in anticoagulant and procoagulant endothelial cell function, consumption in protein C, protein S, and anti-thrombin III, micromeboli, and direct bacterial damage
Diagnosis: severely decreased protein C, protein S, and anti-thrombin III
Clinical: onset usually 7-10 days after the precipitating infection
- Large ecchymoses with sharply demarcated irregular shapes -> evolve into hemorrhagic bullae -> black necrotic lesions and gangrene
- May involve heart, kidneys, and lungs

Fresh Frozen Plasma (FFP) (see Fresh Frozen Plasma): to replace protein C
Low Molecular Weight Heparin/Coumadin (see Enoxaparin and Coumadin): after FFP

Antibiotics: especially to cover for Neisseria and MRSA
Activated Protein C
Intravenous Immunoglobulin (IVIG) (see Intravenous Immunoglobulin): contains antibodies against the causative exotoxins
Surgical Debridement
Hyperbaric Oxygen (see Oxygen): probably not beneficial

Capnocytophaga canimorsus septicemia in Denmark, 1982-1995: review of 39 cases. Clin Infect Dis 1996; 23(1):71-75 [MEDLINE]
Purpura fulminans due to Staphylococcus aureus. Clin Infect Dis. 2005 Apr 1;40(7):941-7 [MEDLINE]
Capnocytophaga canimorsus sepsis. Blood 2010; 116(9):1396 [MEDLINE]
A Case of Purpura Fulminans Arising from Cryptococcosis. Am. J. Respir. Crit. Care Med. 2012; 186: 109-110