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MCID: PRP034
MIFTS: 45

Purpura Fulminans

Categories: Blood diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Purpura Fulminans

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MalaCards integrated aliases for Purpura Fulminans:

Name: Purpura Fulminans 12 55 44 15 73
Purpura Gangrenosa 12

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Blood diseases
See all MalaCards categories (disease lists)
ICD10: 33


External Ids:

Disease Ontology 12 DOID:0060538
ICD10 33 D65
MeSH 44 D055665
UMLS 73 C0085650
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Summaries for Purpura Fulminans

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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 meningococcemia. 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 Protein C and Chlorhexidine have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and endothelial, and related phenotypes are cardiovascular system and embryo

Wikipedia : 76 Purpura fulminans is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising... more...

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Related Diseases for Purpura Fulminans

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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 188)
# Related Disease Score Top Affiliating Genes
1 protein c deficiency 30.5 F2 PROC SERPINC1
2 meningococcemia 30.1 PROC PROCR SERPINC1
3 factor vii deficiency 29.9 F2 F3
4 dengue hemorrhagic fever 29.8 F2 F3
5 osteonecrosis 29.5 F2 SERPINC1
6 endocarditis 29.3 F2 SERPINC1
7 protein s deficiency 29.3 F2 F3 PROS1 SERPINC1
8 hemorrhagic fever 29.1 F2 F3 SERPINC1
9 purpura 28.8 F2 F3 PROC PROCR PROS1 SERPINC1
10 disseminated intravascular coagulation 28.5 F2 F3 SERPINC1 SERPINF2
11 thrombosis 27.8 F2 F3 PROC PROS1 SERPINC1
12 thrombophilia 25.4 F2 F3 PROC PROCR PROS1 SERPINC1
13 acquired purpura fulminans 12.3
14 thrombophilia due to protein c deficiency, autosomal recessive 11.3
15 thrombophilia due to protein s deficiency, autosomal recessive 11.3
16 vitamin k deficiency hemorrhagic disease 10.5 F2 PROS1
17 catastrophic antiphospholipid syndrome 10.4 F3 PROC
18 cerebral falx meningioma 10.4 F2 F3
19 qualitative platelet defect 10.3 F2 F3
20 von willebrand disease, type 1 10.3 F2 F3
21 femoral neuropathy 10.3 F2 F3
22 alcohol-related birth defect 10.3 F2 F3
23 hantavirus pulmonary syndrome 10.3 F2 F3
24 pneumonia 10.3
25 anterior cranial fossa meningioma 10.3 F2 F3
26 splenic disease 10.3 F2 F3
27 hemophilia 10.3 F2 F3
28 endocardium disease 10.3 F2 F3
29 analbuminemia 10.2 F2 F3
30 spinal cord infarction 10.2 F2 SERPINC1
31 squamous cell papilloma 10.2 F2 F3
32 sudden sensorineural hearing loss 10.2 F2 SERPINC1
33 prothrombin deficiency, congenital 10.2 F2 SERPINC1
34 severe hemophilia a 10.2 F2 F3
35 meningitis 10.2
36 ischemic colitis 10.2 F2 SERPINC1
37 anterior spinal artery syndrome 10.2 F2 SERPINC1
38 subendocardial myocardial infarction 10.2 F2 SERPINC1
39 sneddon syndrome 10.2 F2 SERPINC1
40 thrombasthenia 10.2 F2 F3
41 chickenpox 10.2
42 retinal artery occlusion 10.2 F2 SERPINC1
43 esophageal varix 10.2 F2 SERPINC1
44 retinal vascular occlusion 10.2 F2 SERPINC1
45 antithrombin iii deficiency 10.2 F2 SERPINC1
46 post-thrombotic syndrome 10.2 F2 SERPINC1
47 afibrinogenemia, congenital 10.2 F2 F3
48 hypersplenism 10.2 F2 SERPINC1
49 central retinal vein occlusion 10.2 F2 SERPINC1
50 heparin-induced thrombocytopenia 10.2 F3 SERPINC1

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



Diseases related to Purpura Fulminans
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Symptoms & Phenotypes for Purpura Fulminans

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MGI Mouse Phenotypes related to Purpura Fulminans:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.73 PROC PROCR PROS1 SERPINC1 F2 F3
2 embryo MP:0005380 9.55 F3 PROCR PROS1 SERPINC1 F2
3 hematopoietic system MP:0005397 9.43 PROC PROCR PROS1 SERPINC1 F2 F3
4 homeostasis/metabolism MP:0005376 9.17 PROC PROCR PROS1 SERPINC1 SERPINF2 F2
Sources

Drugs & Therapeutics for Purpura Fulminans

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

(show all 25)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Protein C Approved Phase 2, Phase 3
2
Chlorhexidine Approved, Vet_approved Phase 3 55-56-1 9552079 2713
3
Iodine Approved, Investigational Phase 3 7553-56-2 807
4
Povidone Approved Phase 3 9003-39-8
5
Povidone-iodine Approved Phase 3 25655-41-8
6 Anticoagulants Phase 2, Phase 3
7 Fibrinolytic Agents Phase 2, Phase 3
8 Anti-Infective Agents Phase 3
9 Anti-Infective Agents, Local Phase 3
10 Blood Substitutes Phase 3
11 cadexomer iodine Phase 3
12 Chlorhexidine gluconate Phase 3
13 Dermatologic Agents Phase 3
14 Disinfectants Phase 3
15 Micronutrients Phase 3
16 Pharmaceutical Solutions Phase 3
17 Plasma Substitutes Phase 3
18 Trace Elements Phase 3
19
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
20
Butyric Acid Experimental, Investigational Phase 2 107-92-6 264
21 Anti-Inflammatory Agents Phase 2
22 Cortisol succinate Phase 2
23 Hydrocortisone 17-butyrate 21-propionate Phase 2
24 Hydrocortisone acetate Phase 2
25 Hydrocortisone-17-butyrate Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Efficacy and Safety Study of Protein C Concentrate in Subjects With Severe Congenital Protein C Deficiency Completed NCT00157118 Phase 2, Phase 3 Protein C Concentrate (Human) Vapor Heated
2 Study of Chlorhexidine Gluconate as a Preoperative Antisepsis Completed NCT01495117 Phase 3 Povidone-Iodine;Chlorhexidine gluconate
3 Evaluation of Corticosteroid Therapy in Childhood Severe Sepsis - a Randomised Pilot Study Completed NCT00732277 Phase 2 hydrocortisone
4 Outcomes of Purpura FULminans in Adults - The hOPeFUL Study 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

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Genetic Tests for Purpura Fulminans

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Anatomical Context for Purpura Fulminans

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MalaCards organs/tissues related to Purpura Fulminans:

41
Skin, Liver, Endothelial, T Cells, Brain, Bone, Kidney
Sources

Publications for Purpura Fulminans

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Articles related to Purpura Fulminans:

(show top 50) (show all 447)
# Title Authors Year
1
Acquired Versus Congenital Neonatal Purpura Fulminans: A Case Report and Literature Review. ( 29683961 )
2018
2
Purpura fulminans causing acute cortical necrosis in postpartum period. ( 29593006 )
2018
3
An ADAM-10 dependent EPCR shedding links meningococcal interaction with endothelial cells to purpura fulminans. ( 29630665 )
2018
4
A novel compound heterozygous mutations in protein C gene causing neonatal purpura fulminans. ( 29356699 )
2018
5
Two Cases of Israeli Spotted Fever with Purpura Fulminans, Sharon District, Israel. ( 29664383 )
2018
6
Rare presentation of rickettsial infection as purpura fulminans: a case report. ( 29801512 )
2018
7
Acute Kidney Injury in a Case of Purpura Fulminans Developing Secondary to Antithrombin 3 Deficiency. ( 29540648 )
2018
8
Acute infectious purpura fulminans secondary to a dog bite. ( 29437318 )
2018
9
Neonatal Purpura Fulminans, a rare genetic disorder due to protein C deficiency: A case report. ( 29540887 )
2018
10
Purpura fulminans - It's Not Always Sepsis. ( 29381878 )
2018
11
Penile necrosis secondary to purpura fulminans: a case report and review of literature. ( 28479975 )
2017
12
Pathogenesis of meningococcal purpura fulminans. ( 28334263 )
2017
13
Purpura fulminans in congenital protein C deficiency successfully treated with fresh frozen plasma and thrombomodulin. ( 29265490 )
2017
14
Purpura Fulminans: A rare presentation of<i>Streptococcus Pneumoniae</i>infection. ( 29054893 )
2017
15
Mutilating Purpura Fulminans in an Adult with Meningococcal Sepsis. ( 28915308 )
2017
16
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis. ( 29157918 )
2017
17
Skin biopsy polymerase chain reaction for rapid microbiological diagnosis in patients with purpura fulminans. ( 28369801 )
2017
18
Evolution of Purpura Fulminans. ( 28564574 )
2017
19
Purpura fulminans due to pneumococcal infection. ( 29264090 )
2017
20
Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy. ( 28522775 )
2017
21
Purpura Fulminans as the presenting manifestation in a patient with homozygous methylenetetrahydrofolate reductase gene mutation. ( 28612584 )
2017
22
Reconstruction of Near-Total Loss of the Upper and Lower Lips due to Purpura Fulminans with Local Tissue and a Dual-Skin Paddled Anterolateral Thigh Flap. ( 29062669 )
2017
23
Purpura Fulminans in Acute Meningococcemia. ( 28236134 )
2017
24
Purpura Fulminans Associated with Overwhelming Postsplenectomy Infection. ( 28090064 )
2017
25
A novel compound heterozygous form of severe protein C deficiency causing bleeding without purpura fulminans. ( 28485122 )
2017
26
Protein C Deficiency Caused by a Novel Mutation in the<i>PROC</i>Gene in an Infant with Delayed Onset Purpura Fulminans. ( 29082047 )
2017
27
Neisseria meningitidis and purpura fulminans in a woman with chronic liver disease. ( 28346185 )
2017
28
Indian Tick Typhus Presenting as Purpura Fulminans with Review on Rickettsial Infections. ( 28216718 )
2017
29
Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate. ( 29270858 )
2017
30
Challenging Airway Secondary to Purpura Fulminans With Face and Neck Bullae in a Premature Infant: A Case Report. ( 28195860 )
2017
31
Myocarditis and purpura fulminans in meningococcaemia. ( 29024990 )
2017
32
Delayed Surgical Debridement and Use of Semiocclusive Dressings for Salvage of Fingers After Purpura Fulminans. ( 28149224 )
2016
33
Successful Corticosteroid Treatment for Purpura Fulminans Associated with Quinolone. ( 27746448 )
2016
34
Efficacy and safety of protein C concentrate to treat purpura fulminans and thromboembolic events in severe congenital protein C deficiency. ( 27052576 )
2016
35
Purpura fulminans secondary to rickettsial infections: A case series. ( 26955583 )
2016
36
Streptococcus pneumoniae-induced purpura fulminans. ( 27083982 )
2016
37
Purpura fulminans related to paroxysmal nocturnal haemoglobinuria. ( 27046923 )
2016
38
Pneumococcal Sepsis Complicated by Splenic Abscesses and Purpura Fulminans in a 15-Month-Old Child: Case Report and Review of the Literature. ( 27006958 )
2016
39
Acute infectious purpura fulminans caused by group A I^-hemolytic Streptococcus: An uncommon organism. ( 27057503 )
2016
40
Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult. ( 27583208 )
2016
41
Challenging Airway Secondary to Purpura Fulminans With Face and Neck Bullae in a Premature Infant. ( 27941484 )
2016
42
Severe Neonatal Purpura Fulminans Caused by Staphylococcus aureus. ( 27449990 )
2016
43
A Novel Protein C Mutation Causing Neonatal Purpura Fulminans. ( 27889735 )
2016
44
Purpura fulminans associated with acute West Nile virus encephalitis. ( 26686320 )
2016
45
Strongyloides stercoralis hyperinfection in patient with autoimmune hepatitis and purpura fulminans. ( 26955218 )
2016
46
Purpura fulminans and severe sepsis due to Pasteurella multocida infection in an immunocompetent patient. ( 27298290 )
2016
47
Lifetime cost of meningococcal disease in France: Scenarios of severe meningitis and septicemia with purpura fulminans. ( 26688376 )
2016
48
Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia-A Tertiary Center's Experience. ( 26436558 )
2015
49
Angioimmunoblastic t-cell lymphoma presenting as purpura fulminans. ( 25750965 )
2015
50
Purpura Fulminans Due to Acquired Protein C Deficiency. ( 26677306 )
2015
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Variations for Purpura Fulminans

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Expression for Purpura Fulminans

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Search GEO for disease gene expression data for Purpura Fulminans.
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Pathways for Purpura Fulminans

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

# Super pathways Score Top Affiliating Genes
1
Response to elevated platelet cytosolic Ca2+ 66
Show member pathways
 12.78 F2 F3 PROC PROCR PROS1 SERPINC1
2
Collagen chain trimerization 66
Show member pathways
 12.44 F2 F3 PROC PROS1 SERPINC1
3
Cell surface interactions at the vascular wall 66
Show member pathways
 11.92 F2 PROC PROCR PROS1
4
Formation of Fibrin Clot (Clotting Cascade) 66
Show member pathways
 11.7 F2 F3 PROC PROCR PROS1 SERPINC1
5
Gamma carboxylation, hypusine formation and arylsulfatase activation 66
Show member pathways
 11.61 F2 PROC PROS1
6
Complement and coagulation cascades 1 37
11.31 F2 F3 PROC PROCR PROS1 SERPINC1
7
Warfarin Pathway, Pharmacodynamics 61
10.61 F2 PROC PROS1
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GO Terms for Purpura Fulminans

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

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.8 F2 F3 PROCR PROS1 SERPINC1 SERPINF2
2 extracellular space GO:0005615 9.63 F2 F3 PROC PROS1 SERPINC1 SERPINF2
3 endoplasmic reticulum lumen GO:0005788 9.54 F2 PROC SERPINC1
4 Golgi lumen GO:0005796 9.43 F2 PROC PROS1
5 platelet alpha granule lumen GO:0031093 9.4 PROS1 SERPINF2
6 blood microparticle GO:0072562 9.26 F2 PROS1 SERPINC1 SERPINF2
7 extracellular region GO:0005576 9.17 F2 F3 PROC PROCR PROS1 SERPINC1

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.71 F2 PROC SERPINC1
2 ER to Golgi vesicle-mediated transport GO:0006888 9.69 F2 PROC PROS1
3 leukocyte migration GO:0050900 9.67 F2 PROC PROCR PROS1
4 negative regulation of endopeptidase activity GO:0010951 9.63 PROS1 SERPINC1 SERPINF2
5 acute-phase response GO:0006953 9.55 F2 SERPINF2
6 positive regulation of collagen biosynthetic process GO:0032967 9.52 F2 SERPINF2
7 signal peptide processing GO:0006465 9.5 F2 PROC PROS1
8 negative regulation of blood coagulation GO:0030195 9.49 PROC PROS1
9 regulation of blood coagulation GO:0030193 9.48 F2 SERPINC1
10 negative regulation of fibrinolysis GO:0051918 9.43 F2 SERPINF2
11 fibrinolysis GO:0042730 9.43 F2 PROS1 SERPINF2
12 blood coagulation GO:0007596 9.43 F2 F3 PROC PROCR PROS1 SERPINC1
13 negative regulation of coagulation GO:0050819 9.4 PROC PROCR
14 peptidyl-glutamic acid carboxylation GO:0017187 9.33 F2 PROC PROS1
15 hemostasis GO:0007599 9.1 F2 F3 PROC PROCR PROS1 SERPINC1

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

# Name GO ID Score Top Affiliating Genes
1 endopeptidase inhibitor activity GO:0004866 9.16 PROS1 SERPINF2
2 serine-type endopeptidase activity GO:0004252 9.13 F2 F3 PROC
3 protease binding GO:0002020 8.8 F3 SERPINC1 SERPINF2
Sources

Sources for Purpura Fulminans

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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