Purpura fulminans
Encyclopedia
Purpura fulminans is a haemorrhagic condition usually associated with sepsis
or previous infection. It occurs mainly in babies and small children.
It was first described by Guelliot in 1884.
(tissue death), low blood pressure
, fever
and disseminated intravascular coagulation
.
, and other Gram-negative organisms), and deficiency of the natural anticoagulants protein C
or protein S
in the blood. In some cases, a cause is never found.
, escharotomies, fasciotomies, and even amputations. In many cases, digits may need to be amputated when their blood supply has ceased completely.
Drotrecogin alfa
has been recommended for use as ajuvant therapy in some patients.
The use of full dose heparin
or other anticoagulant
is controversial.
It is often difficult to determine tissue viability during the resuscitation phase and debridement and amputation should therefore ideally be deferred until demarcation has taken place.
Sepsis
Sepsis is a potentially deadly medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection. The body may develop this inflammatory response by the immune system to microbes in the blood, urine, lungs, skin, or other tissues...
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
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, 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 pressure
Blood pressure
Blood pressure is the pressure exerted by circulating blood upon the walls of blood vessels, and is one of the principal vital signs. When used without further specification, "blood pressure" usually refers to the arterial pressure of the systemic circulation. During each heartbeat, BP varies...
, fever
Fever
Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
and disseminated intravascular coagulation
Disseminated intravascular coagulation
Disseminated intravascular coagulation , also known as disseminated intravascular coagulopathy or consumptive coagulopathy, is a pathological activation of coagulation mechanisms that happens in response to a variety of diseases. DIC leads to the formation of small blood clots inside the blood...
.
Causes
Common causes are severe infection (especially with meningococcus, and Capnocytophaga canimorsusCapnocytophaga canimorsus
Capnocytophaga canimorsus is a Gram-negative bacillus bacterium that causes a zoonotic disease, most commonly in asplenic patients. It is a member of the normal gingival flora of dogs and cats...
, and other Gram-negative organisms), and deficiency of the natural anticoagulants protein C
Protein C
Protein C, also known as autoprothrombin IIA and blood coagulation factor XIV, is a zymogenic protein, the activated form of which plays an important role in regulating blood clotting, inflammation, cell death and maintaining the permeability of blood vessel walls in humans and other animals...
or protein S
Protein S
Protein 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 humans, protein S is encoded by the PROS1 gene...
in the blood. In some cases, a cause is never found.
Treatment
Treatment is mainly by removing the underlying cause and degree of clotting abnormalities and with supportive treatment (antibiotics, volumne expansion, tissue oxygenation, etc). Thus, treatment includes aggressive management of the septic state. Surgical debridementDebridement
Debridement is the medical removal of a patient's dead, damaged, or infected tissue to improve the healing potential of the remaining healthy tissue...
, escharotomies, fasciotomies, and even amputations. In many cases, digits may need to be amputated when their blood supply has ceased completely.
Drotrecogin alfa
Drotrecogin alfa
Drotrecogin alfa is a recombinant form of human activated protein C that has anti-thrombotic, anti-inflammatory, and profibrinolytic properties. Drotrecogin alpha belongs to the class of serine proteases. Drotrecogin alfa has not been found to improve outcomes in people with severe sepsis...
has been recommended for use as ajuvant therapy in some patients.
The use of full dose heparin
Heparin
Heparin , also known as unfractionated heparin, a highly sulfated glycosaminoglycan, is widely used as an injectable anticoagulant, and has the highest negative charge density of any known biological molecule...
or other anticoagulant
Anticoagulant
An anticoagulant is a substance that prevents coagulation of blood. A group of pharmaceuticals called anticoagulants can be used in vivo as a medication for thrombotic disorders. Some anticoagulants are used in medical equipment, such as test tubes, blood transfusion bags, and renal dialysis...
is controversial.
It is often difficult to determine tissue viability during the resuscitation phase and debridement and amputation should therefore ideally be deferred until demarcation has taken place.