Purpura fulminans
| 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 |
[edit] 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.[citation needed]
[edit] Causes
Common causes are severe infection (especially with meningococcus, and Capnocytophaga canimorsus, and other 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]
[edit] 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.[3] The use of full dose heparin or other anticoagulant is controversial.[citation needed]
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.
[edit] References
- ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ^ Guelliot A (1884). "Note sur trois cas de purpusa infectieux foudroyant". Un Med Sci Nord-Est 8: 25.
- ^ 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.
- ^ 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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