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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

Retiform purpura: Workup and therapeutic considerations in select conditions.
Georgesen C, Fox LP, Harp J
J Am Acad Dermatol 2020 Apr;82(4):799-816. Epub 2019 Aug 31 doi: 10.1016/j.jaad.2019.07.113. PMID: 31479690
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
Genetic Risk Factors in Venous Thromboembolism.
Hotoleanu C
Adv Exp Med Biol 2017;906:253-272. doi: 10.1007/5584_2016_120. PMID: 27638626
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

Idiopathic purpura fulminans.
Sil A, Bhanja DB, Chakraborty S
Postgrad Med J 2020 Aug;96(1138):504. Epub 2019 Dec 4 doi: 10.1136/postgradmedj-2019-137247. PMID: 31801806
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
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

Idiopathic purpura fulminans.
Sil A, Bhanja DB, Chakraborty S
Postgrad Med J 2020 Aug;96(1138):504. Epub 2019 Dec 4 doi: 10.1136/postgradmedj-2019-137247. PMID: 31801806
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
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
Purpura fulminans.
Cram DL, Soley RL
Br J Dermatol 1968 May;80(5):323-7. doi: 10.1111/j.1365-2133.1968.tb12306.x. PMID: 4873077

Prognosis

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
Atypical Presentation Of Rickettsial Spotted Fever.
Prabhakar U, Singh A
J Ayub Med Coll Abbottabad 2017 Oct-Dec;29(4):692-693. PMID: 29331008
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

Vasculitis with nonpalpable purpura resembling purpura fulminans.
Ono E, Takaori K, Maeda S
Clin Exp Nephrol 2020 Feb;24(2):190-191. Epub 2019 Nov 27 doi: 10.1007/s10157-019-01824-4. PMID: 31776714
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

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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