Skip to main content
  • Purpura fulminans has many causes, both infectious and non-infectious.

  • It is a common clinical and histological manifestation of a number of distinct disease processes.

  • The term was first used in the nineteenth century to describe a rapidly progressive skin condition that is most commonly associated with serious systemic infection.

  • Recent years have seen an explosion in our understanding of the pathophysiological processes which occur both in the skin and elsewhere.

  • This has led to advances in management of both the skin condition and underlying disease.

  • We now understand that abnormalities in both the coagulation and fibrinolytic pathways can lead to purpura fulminans.

  • In addition, endothelial cell dysfunction may play a major role in the pathophysiology.

  • Treatments are designed to correct the underlying abnormalities in the coagulation or fibrinolytic pathway, and correct the underlying cause of these abnormalities.

  • Understanding pathophysiology will lead to improve ments in therapeutic modalities and outcome.

  • The prognosis of purpura fulminans has improved in recent years, but is mainly associated with that of the underlying condition.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 149.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Levin M, Eley BS, Faust SN. Purpura Fuminans (Chapter 26.5). In: Harper J, Oranje A, editors. Textbook of Paediatric Dermatology. 2nd Volume. Oxford: Blackwell; 2006. p. 1902–16.

    Google Scholar 

  2. Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost 1990;16(4):299–309.

    Article  PubMed  CAS  Google Scholar 

  3. Francis RB, Jr. Acquired purpura fulminans. Semin Thromb Hemost 1990;16(4):310–25.

    Article  PubMed  Google Scholar 

  4. Levin M, Eley BS, Louis J, et al. Postinfectious purpura fulminans caused by an autoantibody directed against protein S. J Pediatr 1995;127(3):355–63.

    Article  PubMed  CAS  Google Scholar 

  5. Adcock DM, Brozna J, Marlar RA. Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost 1990;16(4):333–40.

    Article  PubMed  CAS  Google Scholar 

  6. Key NS. Toward an understanding of the pathophysiologic mechanism of thrombosis in the antiphospholipid antibody syndrome. J Lab Clin Med 1995;125(1):16–7.

    PubMed  CAS  Google Scholar 

  7. Sotto MN, Langer B, HoshinoShimizu S, de Brito T. Pathogenesis of cutaneous lesions in acute meningococcemia in humans: light, immunofluorescent, and electron microscopic studies of skin biopsy specimens. J Infect Dis 1976;133(5):506–14.

    PubMed  CAS  Google Scholar 

  8. Pathan N, Faust SN, Levin M. Pathophysiology of meningococcal meningitis and septicaemia. Arch Dis Child 2003; 88(7):601–7.

    Article  PubMed  CAS  Google Scholar 

  9. Welch SB, Nadel S. Treatment of meningococcal infection. Arch Dis Child 2003;88(7):608–14.

    Article  PubMed  CAS  Google Scholar 

  10. Booy R, Habibi P, Nadel S, et al. Reduction in case fatality rate from meningococcal disease associated with improved healthcare delivery. Arch Dis Child 2001;85(5):386–90.

    Article  PubMed  CAS  Google Scholar 

  11. Faust SN, Heyderman RS, Levin M. Disseminated intravascular coagulation and purpura fulminans secondary to infection. Baillieres Best Pract Res Clin Haematol 2000;13(2):179–97.

    Article  PubMed  CAS  Google Scholar 

  12. Brandtzaeg P, Sandset PM, Joo GB, et al. The quantitative association of plasma endotoxin, antithrombin, protein C, extrinsic pathway inhibitor and fibrinopeptide A in systemic meningococcal disease. Thromb Res 1989;55 (4):459–70.

    Article  PubMed  CAS  Google Scholar 

  13. Faust SN, Levin M, Harrison OB, et al. Dysfunction of endothelial protein C activation in severe meningococcal sepsis. N Engl J Med 2001;345(6):408–16.

    Article  PubMed  CAS  Google Scholar 

  14. Heyderman RS, Klein NJ, Shennan GI, Levin M. Deficiency of prostacyclin production in meningococcal shock. Arch Dis Child 1991;66(11):1296–9.

    Article  PubMed  CAS  Google Scholar 

  15. Heyderman RS, Klein NJ, Shennan GI, Levin M. Reduction of the anticoagulant activity of glycosaminoglycans on the surface of the vascular endothelium by endotoxin and neutrophils: evaluation by an amidolytic assay. Thromb Res 1992;67(6):677–85.

    Article  PubMed  CAS  Google Scholar 

  16. Osterud B, Flaegstad T. Increased tissue thromboplastin activity in monocytes of patients with meningococcal infection: related to an unfavourable prognosis. Thromb Haemost 1983;49(1):5–7.

    PubMed  CAS  Google Scholar 

  17. Eling M, Stephens AC, Oragui EE, et al. Tissue factor pathway inhibitor (TFPI) levels in the plasma and urine of children with meningococcal disease. Thromb Haemost 2001;85(2):240–4.

    PubMed  CAS  Google Scholar 

  18. Fourrier F, Lestavel P, Chopin C, et al. Meningococcemia and purpura fulminans in adults: acute deficiencies of proteins C and S and early treatment with antithrombin III concentrates. Int Care Med 1990;16(2):121–4.

    Article  CAS  Google Scholar 

  19. Hesselvik JF, Malm J, Dahlback B, Blomback M. Protein C, protein S and C4bbinding protein in severe infection and septic shock. Thromb Haemost 1991;65(2):126–9.

    PubMed  CAS  Google Scholar 

  20. Powars D, Larsen R, Johnson J, et al. Epidemic meningococcemia and purpura fulminans with induced protein C deficiency. Clin Infect Dis 1993;17(2):254–61.

    PubMed  CAS  Google Scholar 

  21. Hermans PW, Hibberd ML, Booy R, et al. 4G/5G promoter polymorphism in the plasminogenactivator-inhibitor-1 gene and outcome of meningococcal disease Meningococcal Research Group. Lancet. 1999;354(9178):556–60.

    Article  PubMed  CAS  Google Scholar 

  22. Westendorp RG, Hottenga JJ, Slagboom PE. Variation in plasminogenactivator-inhibitor-1 gene and risk of meningococcal septic shock. Lancet 1999;354(9178):561–3.

    Article  PubMed  CAS  Google Scholar 

  23. Brandtzaeg P, Joo GB, Brusletto B, Kierulf P. Plasminogen activator inhibitor 1 and 2, alpha-2-antiplasmin, plasminogen, and endotoxin levels in systemic meningococcal disease. Thromb Res 1990;57(2):271–8.

    Article  PubMed  CAS  Google Scholar 

  24. Kornelisse RF, Hazelzet JA, Savelkoul HF, et al. The relationship between plasminogen activator inhibitor-1 and proinflammatory and counterinflammatory mediators in children with meningococcal septic shock. J Infect Dis 1996;173(5):1148–56.

    PubMed  CAS  Google Scholar 

  25. Kondaveeti S, Hibberd ML, Booy R, et al. Effect of the Factor V Leiden mutation on the severity of meningococcal disease. Pediatr Infect Dis J 1999;18(10):893–6.

    Article  PubMed  CAS  Google Scholar 

  26. Pollard AJ, Nadel S, Ninis N, et al. Emergency management of meningococcal disease: eight years on. Arch Dis Child 2007;92(4):283–6.

    Article  PubMed  Google Scholar 

  27. de Jonge E, Levi M, Stoutenbeek CP, van Deventer SJ. Current drug treatment strategies for disseminated intravascular coagulation. Drugs 1998;55(6):767–77.

    Article  PubMed  Google Scholar 

  28. Ockelford P. Heparin 1986. Indications and effective use. Drugs 1986;31(l):81–92.

    Article  PubMed  CAS  Google Scholar 

  29. De Kleijn ED, De Groot R, Hack CE, et al. Activation of protein C following infusion of protein C concentrate in children with severe meningococcal sepsis and purpura fulminans: a randomized, doubleblinded, placebocontrolled, dosefinding study. Crit Care Med 2003;31(6):1839–17.

    Article  PubMed  Google Scholar 

  30. Bernard GR, Vincent JL, Laterre PF, et al. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med 2001;344(10):699–709.

    Article  PubMed  CAS  Google Scholar 

  31. Nadel S, Goldstein B, Williams MD, et al. Drotrecogin alfa (activated) in children with severe sepsis: a multicentre phase III randomised controlled trial. Lancet 2007;369 (9564):836–13.

    Article  PubMed  CAS  Google Scholar 

  32. Warren BL, Eid A, Singer P, et al. Caring for the critically ill patient. Highdose antithrombin III in severe sepsis: a randomized controlled trial. JAMA 2001 17;286(15): 1869–78.

    Article  Google Scholar 

  33. Zenz W, Zöhrer B, Faust SN, et al. Use of tissue plasminogen activator (tPA) in children with fulminant meningococcaemia. Royal College of Paediatrics and Child Health Annual Meeting; 2001;York, UK: RCPCH; 2001.

    Google Scholar 

  34. Davies MS, Nadel S, Habibi P, et al. The orthopaedic management of peripheral ischaemia in meningococcal septicaemia in children. J Bone Joint Surg Br 2000;82(3): 383–6.

    Article  PubMed  CAS  Google Scholar 

  35. Dix D, Andrew M, Marzinotto V, et al. The use of low molecular weight heparin in pediatric patients: a prospective cohort study. J Pediatr 2000;136(4):439–15.

    Article  PubMed  CAS  Google Scholar 

  36. Buysse C, Oranje AP, Zuidema E, Hazelzet JA, Hop W, Diepstraten A, Joosten K. Longterm skin scarring and orthopaedic sequelae in survivors of meningococcal septic shock. Arch Dis Child 2009 [Jan 15, Epub ahead of print]

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Faust, S.N., Nadel, S. (2009). Purpura Fulminans. In: Revuz, J., Roujeau, JC., Kerdel, F.A., Valeyrie-Allanore, L. (eds) Life-Threatening Dermatoses and Emergencies in Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-79339-7_5

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-79339-7_5

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-79338-0

  • Online ISBN: 978-3-540-79339-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics