What is it ?
Purpura fulminans is an infectious syndrome that represents an extremely severe form of sepsis. It causes blood clotting and tissue necrosis. It is very often caused by an invasive meningococcal infection and its outcome is fatal if not managed in time.
Symptoms of Purpura fulminans
High fever, profound damage to the general condition, vomiting and abdominal pain are the first non-characteristic symptoms. One or more red and purple spots spread quickly over the skin, often on the lower limbs. This is purpura, the hemorrhagic lesion of the skin. A pressure on the skin does not flush the blood and does not remove the task momentarily, sign of the “extravasation” of the blood in the tissues. This is due to the fact that Purpura Fulminans causes disseminated intravascular coagulation (DIC), that is, the formation of small clots that will disrupt blood flow ( thrombosis).), leading to the dermis and causing haemorrhage and necrosis of skin tissue. The infectious syndrome may be accompanied by a state of shock or disorders of consciousness of the person affected.
The origins of the disease
In the vast majority of cases, purpura fulminans is linked to an invasive and severe bacterial infection. Neisseria meningitidis (the meningococcus) is the most common infectious agent involved, accounting for approximately 75% of cases. The risk of developing purpura fulminans occurs in 30% of cases of invasive meningococcal disease (IMI).1 to 2 cases of IIM per 100,000 inhabitants occur each year in France, with a fatality rate of about 10%.
Other bacterial agents may be responsible for the development of purpura fulminans, such as Streptococcus pneumoniae (pneumococcus) or Haemophilus influenzae (Pfeiffer bacillus). Sometimes the cause is a deficiency of protein C or S that plays a role in coagulation, due to an inherited genetic abnormality: a mutation of the PROS1 gene (3q11-q11.2) with respect to protein C and PROC gene (2q13-q14) for protein C. It should be noted that purpura fulgurans can result from a mild infection such as chickenpox, in extremely rare cases.
Risk factors for Purpura fulminans
Purpura fulminans can affect any age, but infants less than one year of age and adolescents aged 15 to 20 years are more exposed.People who have been in close contact with the septic shock sufferer should be given prophylaxis to prevent any risk of infection.
Prevention and treatment of Purpura fulminans
The prognosis is directly related to the time of care. Purpura fulminans represents a clinical situation of extreme urgency that requires antibiotic treatment as early as possible, without waiting for the confirmation of the diagnosis and not subject to the preliminary results of a blood culture or a blood test. A purpura consisting of at least one task of diameter greater than or equal to 3 millimeters, must immediately triggers the alert and the treatment. Antibiotic therapy should be appropriate for meningococcal infections and performed intravenously or, alternatively, intramuscularly