Purpura fulminans (also known as "Purpura gangrenosa") is a haemorrhagic condition usually associated with
sepsisSepsis is a serious medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection.
or previous infection. It occurs mainly in babies and small children.
It was first described by Guelliot in 1884.
It is a life-threatening disorder of acute onset. It is characterized by cutaneous haemorrhage and necrosisNecrosis is the premature death of cells and living tissue. Necrosis is caused by external factors, such as infection, toxins , or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
(tissue death), low blood pressureBlood pressure is the pressure exerted by circulating blood on the walls of blood vessels, and is one of the principal vital signs. During each heartbeat, BP varies between a maximum and a minimum pressure...
, feverFever is a frequent medical sign that describes an increase in internal body temperature to levels above normal...
and disseminated intravascular coagulationDisseminated intravascular coagulation , also known as consumptive coagulopathy, is a pathological activation of coagulation mechanisms that happens in response to a variety of diseases. As its name suggests, it leads to the formation of small blood clots inside the blood vessels throughout the body...
.
Common causes are severe infection (especially with meningococcus and Gram-negative organisms), and deficiency of the natural anticoagulants protein CProtein C is a major physiological anticoagulant. It is a vitamin K-dependent serine protease enzyme that is activated by thrombin into activated protein C . The activated form degrades Factor Va and Factor VIIIa...
or protein SProtein S is a vitamin K-dependent plasma glycoprotein synthesized in the endothelium. In the circulation, Protein S exists in two forms: a free form and a complex form bound to complement protein C4b...
in the blood.
Discussion
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Purpura fulminans (also known as "Purpura gangrenosa") is a haemorrhagic condition usually associated with
sepsisSepsis is a serious medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection.
or previous infection. It occurs mainly in babies and small children.
It was first described by Guelliot in 1884.
Presentation
It is a life-threatening disorder of acute onset. It is characterized by cutaneous haemorrhage and necrosisNecrosis is the premature death of cells and living tissue. Necrosis is caused by external factors, such as infection, toxins , or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
(tissue death), low blood pressureBlood pressure is the pressure exerted by circulating blood on the walls of blood vessels, and is one of the principal vital signs. During each heartbeat, BP varies between a maximum and a minimum pressure...
, feverFever is a frequent medical sign that describes an increase in internal body temperature to levels above normal...
and disseminated intravascular coagulationDisseminated intravascular coagulation , also known as consumptive coagulopathy, is a pathological activation of coagulation mechanisms that happens in response to a variety of diseases. As its name suggests, it leads to the formation of small blood clots inside the blood vessels throughout the body...
.
Causes
Common causes are severe infection (especially with meningococcus and Gram-negative organisms), and deficiency of the natural anticoagulants protein CProtein C is a major physiological anticoagulant. It is a vitamin K-dependent serine protease enzyme that is activated by thrombin into activated protein C . The activated form degrades Factor Va and Factor VIIIa...
or protein SProtein S is a vitamin K-dependent plasma glycoprotein synthesized in the endothelium. In the circulation, Protein S exists in two forms: a free form and a complex form bound to complement protein C4b...
in the blood. In some cases, a cause is never found.
Treatment
Treatment is mainly by removing the underlying cause and with supportive treatment. In many cases, digits may need to be amputated when their blood supply has ceased completely.