MCID: PRP034
MIFTS: 43

Purpura Fulminans

Categories: Blood diseases, Rare diseases, Skin diseases
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Aliases & Classifications for Purpura Fulminans

MalaCards integrated aliases for Purpura Fulminans:

Name: Purpura Fulminans 11 53 43 14 16 71 75
Purpura Gangrenosa 11

Classifications:



External Ids:

Disease Ontology 11 DOID:0060538
MeSH 43 D055665
ICD10 31 D65
UMLS 71 C0085650

Summaries for Purpura Fulminans

Disease Ontology: 11 A purpura characterized by blood spots, bruising and discoloration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leading to skin necrosis and disseminated intravascular coagulation. It is often fatal.

MalaCards based summary: Purpura Fulminans, also known as purpura gangrenosa, is related to protein c deficiency and protein s deficiency. An important gene associated with Purpura Fulminans is PROC (Protein C, Inactivator Of Coagulation Factors Va And VIIIa), and among its related pathways/superpathways are Metabolism of proteins and Response to elevated platelet cytosolic Ca2+. The drugs Immunoglobulins and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and endothelial, and related phenotypes are Synthetic lethal with vaccinia virus (VACV) infection and homeostasis/metabolism

Wikipedia: 75 Purpura fulminans is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising... more...

Related Diseases for Purpura Fulminans

Diseases in the Purpura Fulminans family:

Acquired Purpura Fulminans

Diseases related to Purpura Fulminans via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 411)
# Related Disease Score Top Affiliating Genes
1 protein c deficiency 31.6 THBD SERPINC1 PROCR PROC F5 F2
2 protein s deficiency 31.2 THBD SERPINC1 PROS1 PROC F5 F3
3 purpura 31.2 THBD SERPINC1 PROC F3 F2 ADAMTS13
4 thrombocytopenic purpura, autoimmune 30.7 F3 F2 ADAMTS13
5 meningococcemia 30.7 THBD SERPINC1 PROCR F5
6 waterhouse-friderichsen syndrome 30.5 SERPINC1 F3 F2
7 necrotizing fasciitis 30.3 PLG F3 F2
8 pleural empyema 30.3 PLG F2
9 vitamin k deficiency bleeding 30.3 SERPINC1 PROS1 F3 F2
10 compartment syndrome 30.1 SERPINC1 PLG F3 F2
11 limb ischemia 30.0 SERPINC1 PLG F3
12 adult respiratory distress syndrome 30.0 THBD PLG F5
13 anuria 29.9 F3 F2 ADAMTS13
14 leech infestation 29.8 PLG F3 F2 ADAM10
15 aspiration pneumonia 29.8 F3 F2
16 thrombotic thrombocytopenic purpura 29.8 THBD SERPINC1 F3 ADAMTS13
17 hereditary angioedema 29.8 SERPINC1 PLG F2
18 osteonecrosis 29.8 SERPINC1 PLG F2
19 thrombophilia due to thrombin defect 29.6 THBD SERPINC1 PROCR PROC F5 F3
20 deficiency anemia 29.6 SERPINC1 PLG F3 F2 ADAMTS13
21 lemierre's syndrome 29.5 SERPINC1 PLG F3 F2
22 mitral valve stenosis 29.5 SERPINC1 PLG F3 F2
23 varicose veins 29.5 THBD SERPINC1 F5 F2
24 vascular disease 29.5 THBD SERPINC1 F5 F3 F2
25 factor vii deficiency 29.4 TFPI SERPINC1 F3 F2
26 thrombophilia due to activated protein c resistance 29.3 THBD SERPINC1 PLG F5 F3 F2
27 intracranial thrombosis 29.2 SERPINC1 PLG F5 F3 F2
28 antithrombin iii deficiency 29.2 SERPINC1 PLG F5 F3 F2
29 liver cirrhosis 29.2 SERPINF2 SERPINC1 SERPINA1 F3 F2 ADAMTS13
30 respiratory failure 29.1 THBD SERPINC1 SERPINA1 PLG F3 F2
31 disseminated intravascular coagulation 29.0 THBD TFPI SERPINF2 SERPINC1 PROCR PLG
32 afibrinogenemia, congenital 29.0 SERPINF2 SERPINC1 PLG F5 F3 F2
33 systemic lupus erythematosus 28.9 THBD SERPINC1 PROCR PLG F3 F2
34 thrombophlebitis 28.9 SERPINF2 SERPINC1 PROS1 PROC PLG F5
35 lipoprotein quantitative trait locus 28.9 THBD SERPINC1 SERPINA1 PLG F3 F2
36 pulmonary embolism 28.8 THBD SERPINF2 SERPINC1 PROS1 PROC F5
37 factor viii deficiency 28.8 TFPI SERPINC1 SERPINA1 F5 F3 F2
38 malaria 28.7 THBD SERPINC1 SERPINA1 PROCR PLG F3
39 thrombocytopenia 28.6 THBD SERPINC1 PROS1 PROC F5 F3
40 antiphospholipid syndrome 28.6 THBD TFPI SERPINC1 PROCR F5 F3
41 thrombophilia 28.2 THBD TFPI SERPINF2 SERPINC1 PROS1 PROCR
42 thrombosis 27.3 THBD TFPI SERPINF2 SERPINC1 PROS1 PROCR
43 acquired purpura fulminans 11.6
44 thrombophilia due to protein c deficiency, autosomal recessive 11.4
45 thrombophilia due to protein s deficiency, autosomal recessive 11.4
46 toxic shock syndrome 10.8
47 chickenpox 10.8
48 meningococcal infection 10.7
49 exanthem 10.6
50 bacteremia 2 10.5

Graphical network of the top 20 diseases related to Purpura Fulminans:



Diseases related to Purpura Fulminans

Symptoms & Phenotypes for Purpura Fulminans

GenomeRNAi Phenotypes related to Purpura Fulminans according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Synthetic lethal with vaccinia virus (VACV) infection GR00362-A 8.32 SERPINF2

MGI Mouse Phenotypes related to Purpura Fulminans:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.25 ADAM10 ADAMTS13 F2 F3 F5 PLG
2 nervous system MP:0003631 10.2 ADAM10 F2 F3 F5 PLG PROC
3 growth/size/body region MP:0005378 10.1 ADAM10 F2 F3 F5 PLG PROC
4 normal MP:0002873 10.08 ADAM10 ADAMTS13 F2 F3 PROCR PROS1
5 cardiovascular system MP:0005385 10.06 ADAM10 F2 F3 F5 PLG PROC
6 embryo MP:0005380 9.97 ADAM10 F2 F3 F5 PROCR PROS1
7 immune system MP:0005387 9.96 ADAM10 ADAMTS13 F2 F3 PLG PROC
8 hematopoietic system MP:0005397 9.85 ADAM10 ADAMTS13 F2 F3 PLG PROC
9 mortality/aging MP:0010768 9.77 ADAM10 ADAMTS13 F2 F3 F5 PLG
10 integument MP:0010771 9.23 ADAM10 F2 F3 F5 PLG PROS1

Drugs & Therapeutics for Purpura Fulminans

Drugs for Purpura Fulminans (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins
2 Antibodies
3 Protein S

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Short and Long-term Outcomes of Purpura Fulminans in Adults Completed NCT03216577
2 Idiopathic Purpura Fulminans Associated With Anti-PS Antibodies in Children: a French Multicenter Retrospective Study Completed NCT04845113
3 Sepsis-Associated Purpura Fulminans International Registry - Europe Completed NCT02238795
4 Long-term Sequelae of Childhood Meningitis and Meningococcal Purpura Fulminans in Ile de France: a Multidisciplinary Approach Recruiting NCT04685850

Search NIH Clinical Center for Purpura Fulminans

Cochrane evidence based reviews: purpura fulminans

Genetic Tests for Purpura Fulminans

Anatomical Context for Purpura Fulminans

Organs/tissues related to Purpura Fulminans:

MalaCards : Skin, Liver, Endothelial, Uterus, Bone Marrow, Testes, Kidney

Publications for Purpura Fulminans

Articles related to Purpura Fulminans:

(show top 50) (show all 1023)
# Title Authors PMID Year
1
Fulminant sepsis/meningitis due to Haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy. 53 62
18751723 2009
2
Pneumococcal purpura fulminans successfully treated with activated protein C. 53 62
18791506 2008
3
Modern concepts of the diagnosis and treatment of purpura fulminans. 53 62
18652566 2008
4
Hereditary angioedema associated with heterozygous factor V Leiden mutation in a patient with Purpura fulminans. 53 62
17068406 2007
5
Treatment of acute infectious purpura fulminans with activated protein C. 53 62
16820663 2006
6
[Sepsis-associated coagulation disorders]. 53 62
15924156 2005
7
Purpura fulminans due to Staphylococcus aureus. 53 62
15824983 2005
8
Homozygous type I Protein C deficiency in neonatal purpura fulminans with a novel frame-shift deletion of 10 base pairs in exon 8 of PROC gene. 53 62
15748260 2005
9
Massive soft tissue infections: necrotizing fasciitis and purpura fulminans. 53 62
15715517 2005
10
[Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans]. 53 62
15469811 2004
11
Purpura fulminans due to Streptococcus pneumoniae sepsis following gastric bypass. 53 62
15186640 2004
12
Importance of hemostatic gene polymorphisms for susceptibility to and outcome of severe sepsis. 53 62
15118537 2004
13
Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency. 53 62
14707707 2004
14
Combined antithrombin and protein C supplementation in meningococcal purpura fulminans: a pharmacokinetic study. 53 62
12761614 2003
15
Purpura fulminans in a child with combined heterozygous prothrombin G20210A and factor V Leiden mutations. 53 62
12601492 2003
16
Severe protein S deficiency associated with heterozygous factor V Leiden mutation in a child with purpura fulminans. 53 62
12687747 2003
17
[Physiopathology and curative treatment of meningococcal diseases: current aspects]. 53 62
12504371 2002
18
Plasma exchange as a source of protein C for acute onset protein C pathway failure. 53 62
11886399 2002
19
An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia. 53 62
11090052 2000
20
Severe digital necrosis in an elderly patient with heterozygous factor V Leiden mutation. 53 62
11122039 2000
21
A case of purpura fulminans is caused by homozygous delta8857 mutation (protein C-nagoya) and successfully treated with activated protein C concentrate. 53 62
10997987 2000
22
Postinfection purpura fulminans in a patient heterozygous for prothrombin G20210A and acquired protein S resistance. 53 62
10599098 1999
23
Effect of the Factor V Leiden mutation on the severity of meningococcal disease. 53 62
10530586 1999
24
Adult purpura fulminans and digital necrosis associated with sepsis and the factor V mutation. 53 62
9846775 1998
25
Varicella purpura fulminans associated with heterozygosity for factor V leiden and transient protein S deficiency. 53 62
9794956 1998
26
Homozygous Factor V Leiden mutation in a child with meningococcal purpura fulminans. 53 62
9469407 1998
27
[Purpura fulminans in the newborn. Report of two cases successfully treated with heparin and antithrombin III]. 53 62
9577159 1997
28
Purpura fulminans induced by disseminated intravascular coagulation following infection in 2 unrelated children with double heterozygosity for factor V Leiden and protein S deficiency. 53 62
9241737 1997
29
Neonatal purpura fulminans in association with factor V R506Q mutation. 53 62
8627449 1996
30
Meningococcal purpura fulminans treated with antithrombin III concentrate: what is the optimal replacement therapy? 53 62
7848164 1994
31
A homozygous deletion/insertion mutation in the protein C (PROC) gene causing neonatal Purpura fulminans: prenatal diagnosis in an at-risk pregnancy. 53 62
7841323 1994
32
Meningococcemia and purpura fulminans in adults: acute deficiencies of proteins C and S and early treatment with antithrombin III concentrates. 53 62
2139671 1990
33
Panton-Valentine leucocidin-producing Staphylococcus aureus: a clinical review. 62
36040400 2022
34
An Atypical Case of Idiopathic Purpura Fulminans. 62
35700409 2022
35
Two Cases of COVID-19 Patients with Associated Purpura Fulminans. 62
36402743 2022
36
Immunoadsorption therapy for a meningococcemia patient with myocarditis, adrenal hemorrhage, and purpura fulminans: a case report. 62
34284056 2022
37
Purpura fulminans due to Rickettsia japonica. 62
35976146 2022
38
Purpura fulminans in probable scrub typhus group of rickettsioses from hilly region in Nepal - A case report. 62
36268303 2022
39
Multiple free dorsal flaps for symmetrical peripheral gangrene of the extremities: A case report. 62
35591861 2022
40
Purpura fulminans due to Stenotrophomonas maltophilia infection. 62
35123942 2022
41
Meningococcaemia In Children-An Under Recognized Public Health Problem In Pakistan. 62
36414594 2022
42
New-onset diabetic ketoacidosis with purpura fulminans in a child with COVID-19-related multisystem inflammatory syndrome. 62
35300573 2022
43
Kawasaki Disease with Peripheral and Facial Gangrene: A Case report and review of literature. 62
35345947 2022
44
Clustered Incidence of Leukocytoclastic Vasculitis and Purpura Fulminans: A Case Series of a Rare Dermatological Manifestation of Rickettsial Disease. 62
36039228 2022
45
Prevalence, Characteristics, and Determinants of Suboptimal Care in the Initial Management of Community-Onset Severe Bacterial Infections in Children. 62
35696162 2022
46
Purpura fulminans due to Streptococcus pneumoniae bacteraemia in an unsplectomised immunocompetent adult without primary hypocomplementaemia. 62
35589268 2022
47
Prevalence and clinical features of secondary skin lesions in septic patients with bloodstream infections. 62
35320429 2022
48
Routine use of 16S rRNA PCR and subsequent sequencing from blood samples in septic shock: about two case reports of Capnocytophaga canimorsus infection in immunocompetent patients. 62
35397547 2022
49
Purpura fulminans, TEN, and disseminated herpes simplex: An unexpected combination. 62
35498363 2022
50
Severe acquired purpura fulminans in a child. 62
34417189 2022

Variations for Purpura Fulminans

Expression for Purpura Fulminans

Search GEO for disease gene expression data for Purpura Fulminans.

Pathways for Purpura Fulminans

Pathways related to Purpura Fulminans according to GeneCards Suite gene sharing:

(show all 13)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.62 SERPINC1 SERPINA1 PROS1 PROC PLG F5
2
Show member pathways
12.8 THBD TFPI SERPINF2 SERPINC1 SERPINA1 PROS1
3
Show member pathways
12.51 THBD TFPI SERPINC1 PROS1 PROC F5
4
Show member pathways
12.17 ADAM10 F2 F5 PLG PROC SERPINA1
5
Show member pathways
11.96 THBD PROS1 PROCR PROC F2
6
Show member pathways
11.86 THBD TFPI SERPINF2 SERPINC1 SERPINA1 PROS1
7 11.78 PLG F3 ADAM10
8
Show member pathways
11.71 PROS1 PROC F2
9
Show member pathways
11.1 PROS1 PROC F2
10 11.04 TFPI PLG F2
11 10.89 PLG ADAM10
12 10.59 SERPINF2 PLG
13 10.3 PLG F2

GO Terms for Purpura Fulminans

Cellular components related to Purpura Fulminans according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 10.3 ADAMTS13 F2 F3 F5 PLG PROC
2 cell surface GO:0009986 10.22 THBD TFPI SERPINF2 PROCR PLG F3
3 collagen-containing extracellular matrix GO:0062023 10.21 SERPINF2 SERPINC1 SERPINA1 PLG F3 F2
4 blood microparticle GO:0072562 10.07 F2 PLG PROS1 SERPINC1 SERPINF2
5 endoplasmic reticulum lumen GO:0005788 10 SERPINC1 SERPINA1 PROC F5 F2 ADAMTS13
6 serine-type endopeptidase complex GO:1905370 9.88 THBD PROC F2
7 extracellular space GO:0005615 9.74 THBD TFPI SERPINF2 SERPINC1 SERPINA1 PROS1
8 platelet alpha granule lumen GO:0031093 9.65 F5 PLG PROS1 SERPINA1 SERPINF2

Biological processes related to Purpura Fulminans according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 10.2 ADAM10 ADAMTS13 F2 PLG PROC THBD
2 negative regulation of endopeptidase activity GO:0010951 10.1 TFPI SERPINF2 SERPINC1 SERPINA1 PROS1
3 blood coagulation GO:0007596 10.02 ADAMTS13 F2 F3 F5 PLG PROC
4 protein processing GO:0016485 10.01 F3 ADAMTS13 ADAM10
5 acute-phase response GO:0006953 9.95 SERPINF2 SERPINA1 F2
6 fibrinolysis GO:0042730 9.92 F2 PLG PROS1 SERPINF2
7 negative regulation of platelet activation GO:0010544 9.81 THBD F2
8 negative regulation of coagulation GO:0050819 9.8 PROCR PROC
9 negative regulation of blood coagulation GO:0030195 9.8 THBD TFPI PROS1 PROC
10 negative regulation of fibrinolysis GO:0051918 9.76 THBD SERPINF2 PLG F2
11 negative regulation of peptidase activity GO:0010466 9.73 TFPI SERPINF2 SERPINC1 SERPINA1
12 regulation of blood coagulation GO:0030193 9.7 SERPINC1 F2
13 regulation of body fluid levels GO:0050878 9.54 PROC F5 F2
14 hemostasis GO:0007599 9.4 ADAMTS13 F2 F3 F5 PLG PROC

Molecular functions related to Purpura Fulminans according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 10.01 PROC PLG F3 F2
2 protease binding GO:0002020 9.86 F3 SERPINA1 SERPINC1 SERPINF2
3 endopeptidase inhibitor activity GO:0004866 9.85 TFPI SERPINF2 PROS1
4 serine-type endopeptidase inhibitor activity GO:0004867 9.76 TFPI SERPINF2 SERPINC1 SERPINA1
5 peptidase activity GO:0008233 9.72 PROC PLG F2 ADAMTS13 ADAM10
6 endopeptidase activity GO:0004175 9.43 PROC PLG ADAMTS13 ADAM10
7 peptidase inhibitor activity GO:0030414 8.92 TFPI SERPINF2 SERPINC1 SERPINA1

Sources for Purpura Fulminans

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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