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Purpura fulminans
Classification and external resources
ICD-10 D65. (ILDS D65.x20)
ICD-9 286.6
DiseasesDB 34463
MeSH D011695

Purpura fulminans (also known as "Purpura gangrenosa"[1]:825) 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.[2]

Contents

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.{{Fact|date=April 2009}

Causes

Common causes are severe infection (especially with meningococcus and Gram-negative organisms),[3] and deficiency of the natural anticoagulants protein C or protein S in the blood.[4] In some cases, a cause is never found.[3]

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.[3]

References

  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.  
  2. ^ Guelliot A (1884). "Note sur trois cas de purpusa infectieux foudroyant". Un Med Sci Nord-Est 8: 25.  
  3. ^ a b c Nolan J, Sinclair R (2001). "Review of management of purpura fulminans and two case reports". British journal of anaesthesia 86 (4): 581–6. doi:10.1093/bja/86.4.581. PMID 11573639.  
  4. ^ Marciniak E, Wilson HD, Marlar RA (1985). "Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in blood". Blood 65 (1): 15–20. PMID 3838081.  







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