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MCID: PRP034
MIFTS: 45

Purpura Fulminans

Categories: Blood diseases, Rare diseases, Skin 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 56 45 15 17 74
Purpura Gangrenosa 12

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Blood diseases Skin diseases
See all MalaCards categories (disease lists)
ICD10: 34


External Ids:

Disease Ontology 12 DOID:0060538
MeSH 45 D055665
ICD10 34 D65
UMLS 74 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 Iodine have been mentioned in the context of this disorder. Affiliated tissues include skin, endothelial and brain, and related phenotypes are cardiovascular system and embryo

Wikipedia : 77 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 191)
# Related Disease Score Top Affiliating Genes
1 protein c deficiency 30.9 F2 PROC SERPINC1
2 meningococcemia 30.3 PROC PROCR SERPINC1
3 purpura 30.3 F2 F3 PROC PROCR PROS1 SERPINC1
4 catastrophic antiphospholipid syndrome 30.2 F3 PROC
5 protein s deficiency 30.1 F2 F3 PROS1 SERPINC1
6 endocarditis 30.1 F2 SERPINC1
7 antiphospholipid syndrome 30.0 F2 F3 SERPINC1
8 factor vii deficiency 30.0 F2 F3
9 disseminated intravascular coagulation 30.0 F2 F3 SERPINC1 SERPINF2
10 dengue hemorrhagic fever 29.9 F2 F3
11 osteonecrosis 29.8 F2 SERPINC1
12 thrombophilia 28.2 F2 F3 PROC PROCR PROS1 SERPINC1
13 thrombosis 28.2 F2 F3 PROC PROCR PROS1 SERPINC1
14 acquired purpura fulminans 12.5
15 thrombophilia due to protein s deficiency, autosomal recessive 11.8
16 thrombophilia due to protein c deficiency, autosomal recessive 11.5
17 chickenpox 10.5
18 meningitis 10.4
19 malaria 10.3
20 scarlet fever 10.3
21 afibrinogenemia 10.2 F2 F3
22 hemophilia 10.2 F2 F3
23 lupus erythematosus 10.2
24 fasciitis 10.2
25 necrotizing fasciitis 10.2
26 haemophilus influenzae 10.2
27 cerebral falx meningioma 10.2 F2 F3
28 prothrombin deficiency 10.2 F2 F3
29 qualitative platelet defect 10.2 F2 F3
30 femoral neuropathy 10.2 F2 F3
31 alcohol-related birth defect 10.1 F2 F3
32 hantavirus pulmonary syndrome 10.1 F2 F3
33 von willebrand disease, type 1 10.1 F2 F3
34 anterior cranial fossa meningioma 10.1 F2 F3
35 vascular hemostatic disease 10.1 F2 F3
36 splenic disease 10.1 F2 F3
37 retinal artery occlusion 10.1 F2 SERPINC1
38 endocardium disease 10.1 F2 F3
39 spinal cord infarction 10.1 F2 SERPINC1
40 analbuminemia 10.1 F2 F3
41 prothrombin deficiency, congenital 10.1 F2 SERPINC1
42 ischemic colitis 10.1 F2 SERPINC1
43 squamous cell papilloma 10.1 F2 F3
44 factor xiii deficiency 10.1 F3 SERPINF2
45 subendocardial myocardial infarction 10.1 F2 SERPINC1
46 sneddon syndrome 10.1 F2 SERPINC1
47 eclampsia 10.1 F2 SERPINC1
48 systemic lupus erythematosus 10.1
49 arthritis 10.1
50 hepatitis 10.1

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.73 F2 F3 PROC PROCR PROS1 SERPINC1
2 embryo MP:0005380 9.55 F2 F3 PROCR PROS1 SERPINC1
3 hematopoietic system MP:0005397 9.43 F2 F3 PROC PROCR PROS1 SERPINC1
4 homeostasis/metabolism MP:0005376 9.17 F2 F3 PROC PROCR PROS1 SERPINC1
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
Iodine Approved, Investigational Phase 3 7553-56-2 807
3
Povidone Approved Phase 3 9003-39-8
4
Povidone-iodine Approved Phase 3 25655-41-8
5
Chlorhexidine Approved, Vet_approved Phase 3 55-56-1 2713 9552079
6 Anticoagulants Phase 2, Phase 3
7 Fibrinolytic Agents Phase 2, Phase 3
8 Pharmaceutical Solutions Phase 3
9 Dermatologic Agents Phase 3
10 Nutrients Phase 3
11 Disinfectants Phase 3
12 Anti-Infective Agents, Local Phase 3
13 Plasma Substitutes Phase 3
14 Blood Substitutes Phase 3
15 Trace Elements Phase 3
16 cadexomer iodine Phase 3
17 Micronutrients Phase 3
18 Chlorhexidine gluconate Phase 3
19 Anti-Infective Agents Phase 3
20
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
21
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
22 Anti-Inflammatory Agents Phase 2
23 Hydrocortisone-17-butyrate Phase 2
24 Hydrocortisone 17-butyrate 21-propionate Phase 2
25 Hydrocortisone hemisuccinate 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:

42
Skin, Endothelial, Brain, Kidney, T Cells, Spinal Cord, Bone Marrow
Sources

Publications for Purpura Fulminans

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Articles related to Purpura Fulminans:

(show top 50) (show all 490)
# Title Authors Year
1
Four-extremity amputation following disseminated intravascular coagulation and purpura fulminans. ( 30898966 )
2019
2
Meningococcal purpura fulminans and severe myocarditis with clinical meningitis but no meningeal inflammation: a case report. ( 30871501 )
2019
3
Severe cytokine release syndrome resulting in purpura fulminans despite successful response to nivolumab therapy in a patient with pleomorphic carcinoma of the lung: a case report. ( 30944043 )
2019
4
Acute Infectious Purpura Fulminans Caused by Klebsiella oxytoca. ( 30713331 )
2019
5
Acute infectious purpura fulminans with Enterobacter aerogenes post-neurosurgery. ( 30911465 )
2019
6
Management of purpura fulminans skin lesions in a premature neonate with sepsis: a case study. ( 30975056 )
2019
7
A Genetic Origin? Purpura Fulminans. ( 30367859 )
2019
8
Therapeutic response to glucocorticoids, anticoagulation and plasma exchange in a patient with primary antiphospholipid syndrome presenting with purpura fulminans. ( 30290709 )
2018
9
Purpura fulminans-like lesions in antiphospholipid syndrome with endothelial C3 deposition. ( 30364713 )
2018
10
Purpura fulminans with Lemierre's syndrome caused by Gemella bergeri and Eikenella corrodens: a case report. ( 30340466 )
2018
11
Mowat-Wilson Syndrome Presenting With Purpura Fulminans. ( 30573661 )
2018
12
Purpura fulminans in congenital protein C deficiency successfully treated with fresh frozen plasma and thrombomodulin. ( 29265490 )
2018
13
Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate. ( 29270858 )
2018
14
Neonatal Purpura Fulminans, a rare genetic disorder due to protein C deficiency: A case report. ( 29540887 )
2018
15
Purpura fulminans from reduced protein S following cytomegalovirus and varicella infection. ( 30585353 )
2018
16
Sepsis-Associated Purpura Fulminans Due to Klebsiella Oxytoca. ( 30602411 )
2018
17
Escherichia coli Bacteremia-induced Purpura Fulminans: A Case Report. ( 30723639 )
2018
18
Streptococcus pyogenes-purpura fulminans as an invasive form of group A streptococcal infection. ( 29986727 )
2018
19
Clinical spectrum and short-term outcome of adult patients with purpura fulminans: a French multicenter retrospective cohort study. ( 30128591 )
2018
20
Unusual purpura fulminans after heart transplantation. ( 30191293 )
2018
21
Renal histological findings in a patient with acute renal injury associated with purpura fulminans: a case report. ( 30222177 )
2018
22
Purpura Fulminans and Septic Shock due to Capnocytophaga Canimorsus after Dog Bite: A Case Report and Review of the Literature. ( 30225149 )
2018
23
Long-term quality of life in adult patients surviving purpura fulminans: an exposed-unexposed multicenter cohort study. ( 30335142 )
2018
24
Persistence of endothelial thrombomodulin in a patient with infectious purpura fulminans treated with protein C concentrate. ( 30396911 )
2018
25
Purpura fulminans - It's Not Always Sepsis. ( 29381878 )
2018
26
Acute infectious purpura fulminans secondary to a dog bite. ( 29437318 )
2018
27
Acute Kidney Injury in a Case of Purpura Fulminans Developing Secondary to Antithrombin 3 Deficiency. ( 29540648 )
2018
28
Purpura fulminans causing acute cortical necrosis in postpartum period. ( 29593006 )
2018
29
An ADAM-10 dependent EPCR shedding links meningococcal interaction with endothelial cells to purpura fulminans. ( 29630665 )
2018
30
Two Cases of Israeli Spotted Fever with Purpura Fulminans, Sharon District, Israel. ( 29664383 )
2018
31
Acquired Versus Congenital Neonatal Purpura Fulminans: A Case Report and Literature Review. ( 29683961 )
2018
32
Rare presentation of rickettsial infection as purpura fulminans: a case report. ( 29801512 )
2018
33
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis. ( 29157918 )
2018
34
A novel compound heterozygous mutations in protein C gene causing neonatal purpura fulminans. ( 29356699 )
2018
35
Indian Tick Typhus Presenting as Purpura Fulminans with Review on Rickettsial Infections. ( 28216718 )
2017
36
Purpura Fulminans in Acute Meningococcemia. ( 28236134 )
2017
37
Myocarditis and purpura fulminans in meningococcaemia. ( 29024990 )
2017
38
A novel compound heterozygous form of severe protein C deficiency causing bleeding without purpura fulminans. ( 28485122 )
2017
39
Protein C Deficiency Caused by a Novel Mutation in the PROC Gene in an Infant with Delayed Onset Purpura Fulminans. ( 29082047 )
2017
40
Mutilating Purpura Fulminans in an Adult with Meningococcal Sepsis. ( 28915308 )
2017
41
Purpura Fulminans: A rare presentation of Streptococcus Pneumoniae infection. ( 29054893 )
2017
42
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
43
Purpura fulminans due to pneumococcal infection. ( 29264090 )
2017
44
Penile necrosis secondary to purpura fulminans: a case report and review of literature. ( 28479975 )
2017
45
Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy. ( 28522775 )
2017
46
Evolution of Purpura Fulminans. ( 28564574 )
2017
47
Purpura Fulminans as the presenting manifestation in a patient with homozygous methylenetetrahydrofolate reductase gene mutation. ( 28612584 )
2017
48
Purpura Fulminans Associated with Overwhelming Postsplenectomy Infection. ( 28090064 )
2017
49
Challenging Airway Secondary to Purpura Fulminans With Face and Neck Bullae in a Premature Infant: A Case Report. ( 28195860 )
2017
50
Pathogenesis of meningococcal purpura fulminans. ( 28334263 )
2017
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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+ 67
Show member pathways
 12.78 F2 F3 PROC PROCR PROS1 SERPINC1
2
Collagen chain trimerization 67
Show member pathways
 12.44 F2 F3 PROC PROS1 SERPINC1
3
Cell surface interactions at the vascular wall 67
Show member pathways
 11.92 F2 PROC PROCR PROS1
4
Formation of Fibrin Clot (Clotting Cascade) 67
Show member pathways
 11.7 F2 F3 PROC PROCR PROS1 SERPINC1
5
Gamma carboxylation, hypusine formation and arylsulfatase activation 67
Show member pathways
 11.61 F2 PROC PROS1
6
Complement and coagulation cascades 1 38
11.31 F2 F3 PROC PROCR PROS1 SERPINC1
7
Warfarin Pathway, Pharmacodynamics 62
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 PROCR PROS1 SERPINC1 SERPINF2
2 extracellular region GO:0005576 9.7 F2 F3 PROC PROCR PROS1 SERPINC1
3 endoplasmic reticulum lumen GO:0005788 9.54 F2 PROC SERPINC1
4 collagen-containing extracellular matrix GO:0062023 9.5 F3 SERPINC1 SERPINF2
5 platelet alpha granule lumen GO:0031093 9.43 PROS1 SERPINF2
6 Golgi lumen GO:0005796 9.43 F2 PROC PROS1
7 blood microparticle GO:0072562 9.26 F2 PROS1 SERPINC1 SERPINF2
8 extracellular space GO:0005615 9.17 F2 F3 PROC PROCR PROS1 SERPINC1

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.67 F2 PROC SERPINC1
2 ER to Golgi vesicle-mediated transport GO:0006888 9.63 F2 PROC PROS1
3 negative regulation of endopeptidase activity GO:0010951 9.58 PROS1 SERPINC1 SERPINF2
4 acute-phase response GO:0006953 9.51 F2 SERPINF2
5 positive regulation of collagen biosynthetic process GO:0032967 9.48 F2 SERPINF2
6 negative regulation of blood coagulation GO:0030195 9.43 PROC PROS1
7 blood coagulation GO:0007596 9.43 F2 F3 PROC PROCR PROS1 SERPINC1
8 regulation of blood coagulation GO:0030193 9.37 F2 SERPINC1
9 fibrinolysis GO:0042730 9.33 F2 PROS1 SERPINF2
10 negative regulation of fibrinolysis GO:0051918 9.32 F2 SERPINF2
11 negative regulation of coagulation GO:0050819 9.26 PROC PROCR
12 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 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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