MCID: PRP034
MIFTS: 43

Purpura Fulminans

Categories: Blood diseases, Rare diseases, Skin diseases

Aliases & Classifications for Purpura Fulminans

MalaCards integrated aliases for Purpura Fulminans:

Name: Purpura Fulminans 12 54 43 15 17 71
Purpura Gangrenosa 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0060538
MeSH 43 D055665
ICD10 32 D65
UMLS 71 C0085650

Summaries for Purpura Fulminans

Disease Ontology : 12 A purpura characterized blood spots, bruising and discolouration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leads 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 Response to elevated platelet cytosolic Ca2+ and Collagen chain trimerization. The drugs Drotrecogin alfa activated and Anti-Infective Agents have been mentioned in the context of this disorder. Affiliated tissues include skin, brain and liver, and related phenotypes are Decreased replication of vaccinia virus (VACV) and cardiovascular system

Wikipedia : 74 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 350)
# Related Disease Score Top Affiliating Genes
1 protein c deficiency 31.3 THBD SERPINC1 PROCR PROC F5 F2
2 protein s deficiency 31.0 THBD SERPINC1 PROS1 PROC F5 F3
3 compartment syndrome 30.8 SERPINC1 F3 F2
4 waterhouse-friderichsen syndrome 30.7 SERPINC1 F3 F2
5 endocarditis 30.7 SERPINC1 F2 APOH
6 leech infestation 30.7 SERPINC1 F3
7 lemierre's syndrome 30.3 SERPINC1 F3 F2
8 vasculitis 30.3 THBD SERPINA1 APOH
9 mitral valve stenosis 30.3 SERPINC1 F3 F2
10 factor vii deficiency 30.3 SERPINC1 F3 F2
11 anuria 30.3 F3 F2
12 purpura 30.2 THBD SERPINC1 PROS1 PROCR PROC F3
13 meningococcemia 29.9 THBD SERPINC1 PROCR PROC F5
14 raynaud phenomenon 29.8 THBD APOH
15 catastrophic antiphospholipid syndrome 29.7 PROC F5 F3 APOH
16 disseminated intravascular coagulation 29.6 THBD SERPINF2 SERPINC1 PROCR F5 F3
17 hemolytic anemia 29.4 THBD F3 F2 APOH
18 typhoid fever 29.4 THBD SIGLEC5 F2
19 afibrinogenemia, congenital 29.3 SERPINF2 SERPINC1 F5 F3 F2
20 respiratory failure 29.2 THBD SERPINA1 F3 F2
21 thrombotic thrombocytopenic purpura 29.1 THBD SERPINF2 SERPINC1 F3 APOH
22 malaria 29.1 THBD SERPINC1 PROCR F3 F2 APOH
23 thrombocytopenia 29.0 THBD SERPINC1 F5 F3 F2 APOH
24 acute promyelocytic leukemia 28.9 THBD SERPINF2 SERPINC1 F3 F2
25 thrombophlebitis 28.7 SERPINF2 SERPINC1 PROS1 PROC F5 F3
26 antiphospholipid syndrome 28.6 THBD SERPINC1 PROCR F5 F3 F2
27 thrombophilia due to activated protein c resistance 28.5 THBD SERPINF2 SERPINC1 F5 F3 F2
28 thrombophilia due to thrombin defect 28.4 THBD SERPINC1 PROCR PROC F5 F3
29 vascular disease 28.4 THBD SERPINC1 F5 F3 F2 APOH
30 pulmonary embolism 27.7 THBD SERPINF2 SERPINC1 PROS1 PROC F5
31 thrombophilia 27.5 THBD SERPINF2 SERPINC1 PROS1 PROCR PROC
32 thrombosis 27.4 THBD SERPINF2 SERPINC1 PROS1 PROCR PROC
33 acquired purpura fulminans 12.6
34 thrombophilia due to protein c deficiency, autosomal recessive 11.9
35 thrombophilia due to protein s deficiency, autosomal recessive 11.9
36 chickenpox 10.8
37 toxic shock syndrome 10.8
38 exanthem 10.6
39 meningitis 10.5
40 femoral vein thrombophlebitis 10.5 PROC F2
41 neisseria meningitidis infection 10.5
42 achenbach syndrome 10.4 F3 F2
43 acute kidney failure 10.4
44 scarlet fever 10.4
45 synovial angioma 10.4 F3 F2
46 renal pelvis squamous cell carcinoma 10.4 F3 F2
47 papillary adenofibroma 10.4 F3 F2
48 emphysematous cholecystitis 10.4 F3 F2
49 femoral neuropathy 10.4 F3 F2
50 heparin-induced thrombocytopenia 10.4 SERPINC1 F3

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:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased replication of vaccinia virus (VACV) GR00362-A 8.62 PROC THBD

MGI Mouse Phenotypes related to Purpura Fulminans:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 ADAM10 F2 F3 F5 PROC PROCR
2 homeostasis/metabolism MP:0005376 9.9 ADAM10 APOH F2 F3 F5 PROC
3 embryo MP:0005380 9.86 ADAM10 F2 F3 F5 PROCR PROS1
4 mortality/aging MP:0010768 9.7 ADAM10 APOH F2 F3 F5 PROC
5 normal MP:0002873 9.17 ADAM10 F2 F3 PROCR PROS1 SERPINA1

Drugs & Therapeutics for Purpura Fulminans

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

(show all 9)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Drotrecogin alfa activated Phase 3
2 Anti-Infective Agents Phase 3
3
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
4
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
5 Hydrocortisone 17-butyrate 21-propionate Phase 2
6 Hydrocortisone-17-butyrate Phase 2
7 Anti-Inflammatory Agents Phase 2
8 Hydrocortisone hemisuccinate Phase 2
9 Natriuretic Peptide, Brain

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Investigation of the Efficacy and Safety of Drotrecogin Alfa (Activated) in Pediatric Severe Sepsis Completed NCT00049764 Phase 3 Drotrecogin alfa (activated);Placebo
2 Evaluation of Corticosteroid Therapy in Childhood Severe Sepsis (Steroids in Paediatric Sepsis, StePS) - a Randomised Pilot Study Completed NCT00732277 Phase 2 hydrocortisone
3 Septic cArdiac Deficiency and MenIngococcal seveRe Sepsis Unknown status NCT02041663
4 Short and Long-term Outcomes of Purpura Fulminans in Adults Completed NCT03216577
5 Sepsis-Associated Purpura Fulminans International Registry - Europe Recruiting NCT02238795

Search NIH Clinical Center for Purpura Fulminans

Cochrane evidence based reviews: purpura fulminans

Genetic Tests for Purpura Fulminans

Anatomical Context for Purpura Fulminans

MalaCards organs/tissues related to Purpura Fulminans:

40
Skin, Brain, Liver, Endothelial, Bone, Testes, T Cells

Publications for Purpura Fulminans

Articles related to Purpura Fulminans:

(show top 50) (show all 939)
# Title Authors PMID Year
1
Fulminant sepsis/meningitis due to Haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy. 54 61
18751723 2009
2
Pneumococcal purpura fulminans successfully treated with activated protein C. 61 54
18791506 2008
3
Modern concepts of the diagnosis and treatment of purpura fulminans. 54 61
18652566 2008
4
Hereditary angioedema associated with heterozygous factor V Leiden mutation in a patient with Purpura fulminans. 54 61
17068406 2007
5
Treatment of acute infectious purpura fulminans with activated protein C. 61 54
16820663 2006
6
[Sepsis-associated coagulation disorders]. 61 54
15924156 2005
7
Purpura fulminans due to Staphylococcus aureus. 54 61
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. 61 54
15748260 2005
9
Massive soft tissue infections: necrotizing fasciitis and purpura fulminans. 61 54
15715517 2005
10
[Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans]. 61 54
15469811 2004
11
Importance of hemostatic gene polymorphisms for susceptibility to and outcome of severe sepsis. 54 61
15118537 2004
12
Purpura fulminans due to Streptococcus pneumoniae sepsis following gastric bypass. 61 54
15186640 2004
13
Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency. 61 54
14707707 2004
14
Combined antithrombin and protein C supplementation in meningococcal purpura fulminans: a pharmacokinetic study. 54 61
12761614 2003
15
Purpura fulminans in a child with combined heterozygous prothrombin G20210A and factor V Leiden mutations. 61 54
12601492 2003
16
Severe protein S deficiency associated with heterozygous factor V Leiden mutation in a child with purpura fulminans. 54 61
12687747 2003
17
[Physiopathology and curative treatment of meningococcal diseases: current aspects]. 61 54
12504371 2002
18
Plasma exchange as a source of protein C for acute onset protein C pathway failure. 61 54
11886399 2002
19
An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia. 54 61
11090052 2000
20
Severe digital necrosis in an elderly patient with heterozygous factor V Leiden mutation. 54 61
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. 61 54
10997987 2000
22
Postinfection purpura fulminans in a patient heterozygous for prothrombin G20210A and acquired protein S resistance. 61 54
10599098 1999
23
Effect of the Factor V Leiden mutation on the severity of meningococcal disease. 54 61
10530586 1999
24
Adult purpura fulminans and digital necrosis associated with sepsis and the factor V mutation. 54 61
9846775 1998
25
Varicella purpura fulminans associated with heterozygosity for factor V leiden and transient protein S deficiency. 54 61
9794956 1998
26
Homozygous Factor V Leiden mutation in a child with meningococcal purpura fulminans. 54 61
9469407 1998
27
[Purpura fulminans in the newborn. Report of two cases successfully treated with heparin and antithrombin III]. 61 54
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. 54 61
9241737 1997
29
Neonatal purpura fulminans in association with factor V R506Q mutation. 61 54
8627449 1996
30
Meningococcal purpura fulminans treated with antithrombin III concentrate: what is the optimal replacement therapy? 54 61
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. 61 54
7841323 1994
32
Meningococcemia and purpura fulminans in adults: acute deficiencies of proteins C and S and early treatment with antithrombin III concentrates. 61 54
2139671 1990
33
Capnocytophagia canimorsus - Severe sepsis in a previously well individual with no evidence of a cat or dog bite. A case report. 61
32461803 2020
34
Septic Shock and Purpura Fulminans Due to Streptococcus pneumoniae Bacteremia in an Unvaccinated Immunocompetent Adult: Case Report and Review. 61
32513908 2020
35
Epidemiology of Community-Onset Severe Bacterial Infections in Children and Its Evolution: A Population-Based Study in France. 61
32224829 2020
36
[Acute fever with rash, necrosis, and bullae in both lower extremities in a 12-year-old girl]. 61
32571467 2020
37
Acute Purpura Fulminans - a rare cause of skin necrosis: A single institution clinicopathological experience. 61
32356378 2020
38
[Treatment failure after postinfectious purpura fulminans despite interdisciplinary surgical and intensive medical therapy]. 61
32055865 2020
39
Purpuric Epiglottic Lesion in Purpura Fulminans Form of Meningococcemia. 61
32293909 2020
40
Anti-interleukin-6 receptor therapy-induced cutaneous symptoms resembling purpura fulminans in a patient with seropositive rheumatoid arthritis. 61
32277528 2020
41
Retiform purpura: Workup and therapeutic considerations in select conditions. 61
31479690 2020
42
Meningococcal disease: A paradigm of type-IV pilus dependent pathogenesis. 61
32185901 2020
43
Neisseria meningitidis Induced Fatal Waterhouse-Friderichsen Syndrome in a Patient Presenting With Disseminated Intravascular Coagulation and Multiple Organ Failure. 61
32192003 2020
44
Pneumococcal Sepsis-Induced Purpura Fulminans in an Asplenic Adult Patient with Disseminated Intravascular Coagulation. 61
32223817 2020
45
Successful treatment of meningococcal bacteremia using oral doxycycline: A case report. 61
31887457 2020
46
Clinical manifestations of invasive meningococcal disease in Victoria with the emergence of serogroup W and serogroup Y Neisseria meningitidis. 61
32043702 2020
47
Pneumococcal purpura fulminans in asplenic or hyposplenic patients: a French multicenter exposed-unexposed retrospective cohort study. 61
32102696 2020
48
Vasculitis with nonpalpable purpura resembling purpura fulminans. 61
31776714 2020
49
Development of Bilateral Heterotopic Ossification After Survival of Life Threatening Purpura Fulminans. 61
32133255 2020
50
Purpura Fulminans: a Rare but Fierce Presentation of Pneumococcal Sepsis. 61
32015972 2020

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.97 THBD SERPINF2 SERPINC1 SERPINA1 PROS1 PROCR
2
Show member pathways
12.53 THBD SERPINC1 PROS1 PROC F5 F3
3
Show member pathways
11.97 THBD PROS1 PROCR PROC F2
4
Show member pathways
11.81 THBD SERPINF2 SERPINC1 PROS1 PROCR PROC
5
Show member pathways
11.61 PROS1 PROC F2
6 11.48 THBD SERPINF2 SERPINC1 SERPINA1 PROS1 PROCR
7 10.72 PROS1 PROC 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 exosome GO:0070062 10.03 SERPINF2 SERPINC1 SERPINA1 PROS1 PROCR F2
2 extracellular region GO:0005576 10.02 SERPINF2 SERPINC1 SERPINA1 PROS1 PROCR PROC
3 cell surface GO:0009986 9.85 THBD SERPINF2 PROCR F3 APOH ADAM10
4 blood microparticle GO:0072562 9.71 SERPINF2 SERPINC1 PROS1 F2
5 Golgi lumen GO:0005796 9.63 PROS1 PROC F2
6 collagen-containing extracellular matrix GO:0062023 9.63 SERPINF2 SERPINC1 SERPINA1 F3 F2 APOH
7 platelet alpha granule lumen GO:0031093 9.56 SERPINF2 SERPINA1 PROS1 F5
8 endoplasmic reticulum lumen GO:0005788 9.43 SERPINC1 SERPINA1 PROC F5 F2 ADAM10
9 extracellular space GO:0005615 9.36 THBD SERPINF2 SERPINC1 SERPINA1 PROS1 PROCR

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

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 post-translational protein modification GO:0043687 9.88 SERPINC1 SERPINA1 PROC F5 ADAM10
2 ER to Golgi vesicle-mediated transport GO:0006888 9.83 SERPINA1 PROS1 PROC F5 F2
3 negative regulation of endopeptidase activity GO:0010951 9.81 SERPINF2 SERPINC1 SERPINA1 PROS1
4 cellular protein metabolic process GO:0044267 9.8 SERPINC1 SERPINA1 PROC F5 F2 ADAM10
5 platelet degranulation GO:0002576 9.77 SERPINF2 SERPINA1 PROS1 F5 APOH
6 negative regulation of peptidase activity GO:0010466 9.73 SERPINF2 SERPINC1 SERPINA1
7 acute-phase response GO:0006953 9.67 SERPINF2 SERPINA1 F2
8 fibrinolysis GO:0042730 9.63 SERPINF2 PROS1 F2
9 blood coagulation GO:0007596 9.61 THBD SERPINC1 SERPINA1 PROS1 PROCR PROC
10 positive regulation of collagen biosynthetic process GO:0032967 9.58 SERPINF2 F2
11 regulation of blood coagulation GO:0030193 9.58 SERPINC1 F2 APOH
12 blood coagulation, intrinsic pathway GO:0007597 9.57 F2 APOH
13 negative regulation of blood coagulation GO:0030195 9.56 THBD PROS1 PROC APOH
14 positive regulation of blood coagulation GO:0030194 9.55 F2 APOH
15 negative regulation of platelet activation GO:0010544 9.54 THBD F2
16 negative regulation of coagulation GO:0050819 9.52 PROCR PROC
17 negative regulation of fibrinolysis GO:0051918 9.46 THBD SERPINF2 F2 APOH
18 hemostasis GO:0007599 9.28 THBD SERPINC1 SERPINA1 PROS1 PROCR PROC

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.18 THBD SIGLEC5 SERPINF2 SERPINC1 SERPINA1 PROCR
2 serine-type endopeptidase activity GO:0004252 9.5 PROC F3 F2
3 heparin binding GO:0008201 9.43 SERPINC1 F2 APOH
4 peptidase inhibitor activity GO:0030414 9.33 SERPINF2 SERPINC1 SERPINA1
5 serine-type endopeptidase inhibitor activity GO:0004867 9.13 SERPINF2 SERPINC1 SERPINA1
6 protease binding GO:0002020 8.92 SERPINF2 SERPINC1 SERPINA1 F3

Sources for Purpura Fulminans

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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