Purpura fulminans

Purpura fulminans (also known as "Purpura gangrenosa" ) 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.

Presentation
It is a life-threatening disorder of acute onset. It is characterized by cutaneous haemorrhage and necrosis (tissue death), low blood pressure, fever and disseminated intravascular coagulation.

Causes
Common causes are severe infection (especially with meningococcus, and Capnocytophaga canimorsus, 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.

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 debridement, 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.