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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

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
Activated protein C.
Griffin JH, Fernández JA, Gale AJ, Mosnier LO
J Thromb Haemost 2007 Jul;5 Suppl 1:73-80. doi: 10.1111/j.1538-7836.2007.02491.x. PMID: 17635713

Diagnosis

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
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

Therapy

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
Anticoagulants in dermatology.
Adya KA, Inamadar AC, Palit A
Indian J Dermatol Venereol Leprol 2016 Nov-Dec;82(6):626-640. doi: 10.4103/0378-6323.184199. PMID: 27320765
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
Pigmented purpura and cutaneous vascular occlusion syndromes.
Lamadrid-Zertuche AC, Garza-Rodríguez V, Ocampo-Candiani JJ
An Bras Dermatol 2018 Jun;93(3):397-404. doi: 10.1590/abd1806-4841.20187459. PMID: 29924250Free 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

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
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
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
Coagulopathy of sepsis.
Dempfle CE
Thromb Haemost 2004 Feb;91(2):213-24. doi: 10.1160/TH03-03-0182. PMID: 14961146

Recent systematic reviews

Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review.
Theron A, Dautremay O, Boissier E, Zerroukhi A, Baleine J, Moulis L, Rodière M, Schved JF, Duraes M, Kanouni T, Cau-Diaz I, Jeziorski E, Biron-Andreani C
Blood Adv 2022 Jan 25;6(2):495-502. doi: 10.1182/bloodadvances.2021005126. PMID: 34788405Free PMC Article

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