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Purpura fulminans

MedGen UID:
88448
•Concept ID:
C0085650
•
Disease or Syndrome
Synonyms: Fulminans, Purpura; Purpura Fulminans
SNOMED CT: Purpura fulminans (13507004)
 
Monarch Initiative: MONDO:0000809

Definition

A severe, rapidly fatal reaction occurring most commonly in children following an infectious illness. It is characterized by large, rapidly spreading skin hemorrhages, fever, or shock. Purpura fulminans often accompanies or is triggered by disseminated intravascular coagulation. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPurpura fulminans

Professional guidelines

PubMed

Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Purpura fulminans: recognition, diagnosis and management.
Chalmers E, Cooper P, Forman K, Grimley C, Khair K, Minford A, Morgan M, Mumford AD
Arch Dis Child 2011 Nov;96(11):1066-71. Epub 2011 Jan 12 doi: 10.1136/adc.2010.199919. PMID: 21233082
Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.
Levi M, Toh CH, Thachil J, Watson HG
Br J Haematol 2009 Apr;145(1):24-33. Epub 2009 Feb 12 doi: 10.1111/j.1365-2141.2009.07600.x. PMID: 19222477

Recent clinical studies

Etiology

Panton-Valentine leucocidin-producing Staphylococcus aureus: a clinical review.
Hussain K, Bandyopadhyay A, Roberts N, Mughal N, Moore LSP, Fuller LC
Clin Exp Dermatol 2022 Dec;47(12):2150-2158. Epub 2022 Oct 11 doi: 10.1111/ced.15392. PMID: 36040400
Protein C Deficiency.
Dinarvand P, Moser KA
Arch Pathol Lab Med 2019 Oct;143(10):1281-1285. Epub 2019 Feb 1 doi: 10.5858/arpa.2017-0403-RS. PMID: 30702334
American Society of Hematology 2018 Guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism.
Monagle P, Cuello CA, Augustine C, Bonduel M, Brandão LR, Capman T, Chan AKC, Hanson S, Male C, Meerpohl J, Newall F, O'Brien SH, Raffini L, van Ommen H, Wiernikowski J, Williams S, Bhatt M, Riva JJ, Roldan Y, Schwab N, Mustafa RA, Vesely SK
Blood Adv 2018 Nov 27;2(22):3292-3316. doi: 10.1182/bloodadvances.2018024786. PMID: 30482766Free PMC Article
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.
Levi M, Toh CH, Thachil J, Watson HG
Br J Haematol 2009 Apr;145(1):24-33. Epub 2009 Feb 12 doi: 10.1111/j.1365-2141.2009.07600.x. PMID: 19222477

Diagnosis

Panton-Valentine leucocidin-producing Staphylococcus aureus: a clinical review.
Hussain K, Bandyopadhyay A, Roberts N, Mughal N, Moore LSP, Fuller LC
Clin Exp Dermatol 2022 Dec;47(12):2150-2158. Epub 2022 Oct 11 doi: 10.1111/ced.15392. PMID: 36040400
Protein C Deficiency.
Dinarvand P, Moser KA
Arch Pathol Lab Med 2019 Oct;143(10):1281-1285. Epub 2019 Feb 1 doi: 10.5858/arpa.2017-0403-RS. PMID: 30702334
Neonatal Skin Emergencies.
Siegel M, Lee LW
Pediatr Ann 2019 Jan 1;48(1):e36-e42. doi: 10.3928/19382359-20181210-03. PMID: 30653641
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Purpura fulminans: recognition, diagnosis and management.
Chalmers E, Cooper P, Forman K, Grimley C, Khair K, Minford A, Morgan M, Mumford AD
Arch Dis Child 2011 Nov;96(11):1066-71. Epub 2011 Jan 12 doi: 10.1136/adc.2010.199919. PMID: 21233082

Therapy

Thrombosis questions from the inpatient wards.
Goshua G, Bendapudi PK, Lee AI
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):481-490. doi: 10.1182/hematology.2022000384. PMID: 36485139Free PMC Article
American Society of Hematology 2018 Guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism.
Monagle P, Cuello CA, Augustine C, Bonduel M, Brandão LR, Capman T, Chan AKC, Hanson S, Male C, Meerpohl J, Newall F, O'Brien SH, Raffini L, van Ommen H, Wiernikowski J, Williams S, Bhatt M, Riva JJ, Roldan Y, Schwab N, Mustafa RA, Vesely SK
Blood Adv 2018 Nov 27;2(22):3292-3316. doi: 10.1182/bloodadvances.2018024786. PMID: 30482766Free PMC Article
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Purpura fulminans: recognition, diagnosis and management.
Chalmers E, Cooper P, Forman K, Grimley C, Khair K, Minford A, Morgan M, Mumford AD
Arch Dis Child 2011 Nov;96(11):1066-71. Epub 2011 Jan 12 doi: 10.1136/adc.2010.199919. PMID: 21233082
Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.
Levi M, Toh CH, Thachil J, Watson HG
Br J Haematol 2009 Apr;145(1):24-33. Epub 2009 Feb 12 doi: 10.1111/j.1365-2141.2009.07600.x. PMID: 19222477

Prognosis

Skin and sepsis: contribution of dermatology to a rapid diagnosis.
Pulido-Pérez A, Bergón-Sendín M, Suárez-Fernández R, Muñoz-Martín P, Bouza E
Infection 2021 Aug;49(4):617-629. Epub 2021 Apr 15 doi: 10.1007/s15010-021-01608-7. PMID: 33860474
Protein C Deficiency.
Dinarvand P, Moser KA
Arch Pathol Lab Med 2019 Oct;143(10):1281-1285. Epub 2019 Feb 1 doi: 10.5858/arpa.2017-0403-RS. PMID: 30702334
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
Colling ME, Bendapudi PK
Transfus Med Rev 2018 Apr;32(2):69-76. Epub 2017 Oct 16 doi: 10.1016/j.tmrv.2017.10.001. PMID: 29157918
Microparticles and infectious diseases.
Delabranche X, Berger A, Boisramé-Helms J, Meziani F
Med Mal Infect 2012 Aug;42(8):335-43. Epub 2012 Jul 4 doi: 10.1016/j.medmal.2012.05.011. PMID: 22766273
Purpura fulminans: recognition, diagnosis and management.
Chalmers E, Cooper P, Forman K, Grimley C, Khair K, Minford A, Morgan M, Mumford AD
Arch Dis Child 2011 Nov;96(11):1066-71. Epub 2011 Jan 12 doi: 10.1136/adc.2010.199919. PMID: 21233082

Clinical prediction guides

The rash that becomes purpuric, petechial, hemorrhagic, or ecchymotic.
Antonov D, Kamarashev J, Kazandjieva J, Neykova T, Tsankov N
Clin Dermatol 2020 Jan-Feb;38(1):3-18. Epub 2019 Aug 1 doi: 10.1016/j.clindermatol.2019.07.036. PMID: 32197746
Pathogenesis of meningococcemia.
Coureuil M, Join-Lambert O, Lécuyer H, Bourdoulous S, Marullo S, Nassif X
Cold Spring Harb Perspect Med 2013 Jun 1;3(6) doi: 10.1101/cshperspect.a012393. PMID: 23732856Free PMC Article
Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.
Levi M, Toh CH, Thachil J, Watson HG
Br J Haematol 2009 Apr;145(1):24-33. Epub 2009 Feb 12 doi: 10.1111/j.1365-2141.2009.07600.x. PMID: 19222477
Coagulation in sepsis.
Jagneaux T, Taylor DE, Kantrow SP
Am J Med Sci 2004 Oct;328(4):196-204. doi: 10.1097/00000441-200410000-00002. PMID: 15486534
Coagulopathy of sepsis.
Dempfle CE
Thromb Haemost 2004 Feb;91(2):213-24. doi: 10.1160/TH03-03-0182. PMID: 14961146

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