Purpura fulminans

Purpura fulminans

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. [cite journal|author=Guelliot A|year=1884|title=Note sur trois cas de purpusa infectieux foudroyant|journal=Un Med Sci Nord-Est|volume=8|pages=25]

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 Gram-negative organisms),cite journal |author=Nolan J, Sinclair R |title=Review of management of purpura fulminans and two case reports |journal=British journal of anaesthesia |volume=86 |issue=4 |pages=581–6 |year=2001 |pmid=11573639 |doi=] and deficiency of the natural anticoagulants protein C or protein S in the blood. [cite journal |author=Marciniak E, Wilson HD, Marlar RA |title=Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in blood |journal=Blood |volume=65 |issue=1 |pages=15–20 |year=1985 |pmid=3838081 |doi=] 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.

References


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