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Arch Dis Child 96:1066-1071 doi:10.1136/adc.2010.199919
  • Reviews

Purpura fulminans: recognition, diagnosis and management

  1. AD Mumford7
  1. 1Haemophilia Centre, Yorkhill Children's Hospital, Glasgow, UK
  2. 2Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, UK
  3. 3Department of Haematology, Nottingham University Hospital, Nottingham, UK
  4. 4Haemophilia Centre, Great Ormond Street Hospital for Children NHS Trust, London, UK
  5. 5Department of Paediatrics, Bradford Royal Infirmary, Bradford, UK
  6. 6Department of Paediatric Oncology and Haematology, Southampton General Hospital, Southampton, UK
  7. 7Bristol Heart Institute, University of Bristol, Bristol, UK
  1. Correspondence to Dr Andrew Mumford
    Bristol Heart Institute, University of Bristol, Level 7 Bristol Royal Infirmary, Bristol BS2 8HW, UK; a.mumford{at}bristol.ac.uk
  • Accepted 17 November 2010
  • Published Online First 12 January 2011

Abstract

Purpura fulminans (PF) is a haematological emergency in which there is skin necrosis and disseminated intravascular coagulation. This may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels. PF may complicate severe sepsis or may occur as an autoimmune response to otherwise benign childhood infections. PF may also be the presenting symptom of severe heritable deficiency of the natural anticoagulants protein C or protein S. Early recognition and treatment of PF is essential to reduce mortality and to prevent major long-term health sequelae. However, management strategies require accurate identification of the underlying cause. This review focuses on the clinical features, differential diagnosis and laboratory features of the range of PF disorders and includes expert consensus opinion about immediate and on-going management.

Footnotes

  • Funding The clinical expert panel meetings from which this guidance document was generated were supported by an unrestricted educational grant from Baxter Healthcare UK.

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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