MCID: PRP034
MIFTS: 44

Purpura Fulminans

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Purpura Fulminans

MalaCards integrated aliases for Purpura Fulminans:

Name: Purpura Fulminans 12 51 41 14 69
Purpura Gangrenosa 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0060538
ICD10 32 D65
MeSH 41 D055665
UMLS 69 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 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 Anticoagulants have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and t cells, and related phenotypes are cardiovascular system and embryo

Wikipedia : 72 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 189)
# Related Disease Score Top Affiliating Genes
1 protein c deficiency 30.6 F2 PROC SERPINC1
2 meningococcemia 30.2 PROC PROCR SERPINC1
3 purpura 29.9 F2 F3 PROC PROCR PROS1 SERPINC1
4 factor vii deficiency 29.8 F2 F3
5 protein s deficiency 29.8 F2 F3 PROS1 SERPINC1
6 dengue hemorrhagic fever 29.7 F2 F3
7 osteonecrosis 29.6 F2 SERPINC1
8 endocarditis 29.5 F2 SERPINC1
9 hemorrhagic fever 29.3 F2 F3 SERPINC1
10 disseminated intravascular coagulation 29.2 F2 F3 SERPINC1 SERPINF2
11 thrombosis 28.5 F2 F3 PROC PROS1 SERPINC1
12 thrombophilia 27.0 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 pneumonia 10.3
17 vitamin k deficiency hemorrhagic disease 10.3 F2 PROS1
18 catastrophic antiphospholipid syndrome 10.3 F3 PROC
19 meningitis 10.2
20 cerebral falx meningioma 10.2 F2 F3
21 von willebrand disease, type 1 10.2 F2 F3
22 qualitative platelet defect 10.2 F2 F3
23 femoral neuropathy 10.2 F2 F3
24 alcohol-related birth defect 10.2 F2 F3
25 hantavirus pulmonary syndrome 10.2 F2 F3
26 anterior cranial fossa meningioma 10.2 F2 F3
27 chickenpox 10.2
28 splenic disease 10.2 F2 F3
29 factor v deficiency 10.2 F2 F3
30 squamous cell papilloma 10.2 F2 F3
31 hemophilia 10.2 F2 F3
32 analbuminemia 10.1 F2 F3
33 endocardium disease 10.1 F2 F3
34 spinal cord infarction 10.1 F2 SERPINC1
35 sudden sensorineural hearing loss 10.1 F2 SERPINC1
36 prothrombin deficiency, congenital 10.1 F2 SERPINC1
37 ischemic colitis 10.1 F2 SERPINC1
38 severe hemophilia a 10.1 F2 F3
39 anterior spinal artery syndrome 10.1 F2 SERPINC1
40 malaria 10.1
41 burns 10.1
42 subendocardial myocardial infarction 10.1 F2 SERPINC1
43 sneddon syndrome 10.1 F2 SERPINC1
44 thrombasthenia 10.1 F2 F3
45 retinal artery occlusion 10.1 F2 SERPINC1
46 esophageal varix 10.1 F2 SERPINC1
47 retinal vascular occlusion 10.1 F2 SERPINC1
48 antithrombin iii deficiency 10.1 F2 SERPINC1
49 post-thrombotic syndrome 10.1 F2 SERPINC1
50 intracranial sinus thrombosis 10.1 F3 SERPINC1

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



Diseases related to Purpura Fulminans

Symptoms & Phenotypes for Purpura Fulminans

MGI Mouse Phenotypes related to Purpura Fulminans:

43
# 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

Drugs & Therapeutics for Purpura Fulminans

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

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Protein C Approved Phase 2, Phase 3
2 Anticoagulants Phase 2, Phase 3
3 Fibrinolytic Agents Phase 2, Phase 3
4
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 657311 5754
5
Butyric Acid Experimental, Investigational Phase 2 107-92-6 264
6 Anti-Inflammatory Agents Phase 2
7 Cortisol succinate Phase 2
8 Hydrocortisone 17-butyrate 21-propionate Phase 2
9 Hydrocortisone acetate Phase 2
10 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 Evaluation of Corticosteroid Therapy in Childhood Severe Sepsis - a Randomised Pilot Study Completed NCT00732277 Phase 2 hydrocortisone
3 Outcomes of Purpura FULminans in Adults - The hOPeFUL Study Recruiting NCT03216577
4 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:

38
Skin, Liver, T Cells, Brain, Breast, Bone

Publications for Purpura Fulminans

Articles related to Purpura Fulminans:

(show top 50) (show all 440)
# Title Authors Year
1
Acute infectious purpura fulminans secondary to a dog bite. ( 29437318 )
2018
2
Purpura fulminans - It's Not Always Sepsis. ( 29381878 )
2018
3
A novel compound heterozygous mutations in protein C gene causing neonatal purpura fulminans. ( 29356699 )
2018
4
Indian Tick Typhus Presenting as Purpura Fulminans with Review on Rickettsial Infections. ( 28216718 )
2017
5
Skin biopsy polymerase chain reaction for rapid microbiological diagnosis in patients with purpura fulminans. ( 28369801 )
2017
6
Evolution of Purpura Fulminans. ( 28564574 )
2017
7
Purpura Fulminans Associated with Overwhelming Postsplenectomy Infection. ( 28090064 )
2017
8
Purpura Fulminans as the presenting manifestation in a patient with homozygous methylenetetrahydrofolate reductase gene mutation. ( 28612584 )
2017
9
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
10
A novel compound heterozygous form of severe protein C deficiency causing bleeding without purpura fulminans. ( 28485122 )
2017
11
Penile necrosis secondary to purpura fulminans: a case report and review of literature. ( 28479975 )
2017
12
Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate. ( 29270858 )
2017
13
Purpura Fulminans: A rare presentation of<i>Streptococcus Pneumoniae</i>infection. ( 29054893 )
2017
14
Challenging Airway Secondary to Purpura Fulminans With Face and Neck Bullae in a Premature Infant: A Case Report. ( 28195860 )
2017
15
Mutilating Purpura Fulminans in an Adult with Meningococcal Sepsis. ( 28915308 )
2017
16
Purpura Fulminans in Acute Meningococcemia. ( 28236134 )
2017
17
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis. ( 29157918 )
2017
18
Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy. ( 28522775 )
2017
19
Myocarditis and purpura fulminans in meningococcaemia. ( 29024990 )
2017
20
Purpura fulminans in congenital protein C deficiency successfully treated with fresh frozen plasma and thrombomodulin. ( 29265490 )
2017
21
Pathogenesis of meningococcal purpura fulminans. ( 28334263 )
2017
22
Purpura fulminans due to pneumococcal infection. ( 29264090 )
2017
23
Neisseria meningitidis and purpura fulminans in a woman with chronic liver disease. ( 28346185 )
2017
24
Protein C Deficiency Caused by a Novel Mutation in the<i>PROC</i>Gene in an Infant with Delayed Onset Purpura Fulminans. ( 29082047 )
2017
25
A Novel Protein C Mutation Causing Neonatal Purpura Fulminans. ( 27889735 )
2016
26
Purpura fulminans secondary to rickettsial infections: A case series. ( 26955583 )
2016
27
Successful Corticosteroid Treatment for Purpura Fulminans Associated with Quinolone. ( 27746448 )
2016
28
Pneumococcal Sepsis Complicated by Splenic Abscesses and Purpura Fulminans in a 15-Month-Old Child: Case Report and Review of the Literature. ( 27006958 )
2016
29
Strongyloides stercoralis hyperinfection in patient with autoimmune hepatitis and purpura fulminans. ( 26955218 )
2016
30
Purpura fulminans associated with acute West Nile virus encephalitis. ( 26686320 )
2016
31
Lifetime cost of meningococcal disease in France: Scenarios of severe meningitis and septicemia with purpura fulminans. ( 26688376 )
2016
32
Streptococcus pneumoniae-induced purpura fulminans. ( 27083982 )
2016
33
Purpura fulminans and severe sepsis due to Pasteurella multocida infection in an immunocompetent patient. ( 27298290 )
2016
34
Purpura fulminans related to paroxysmal nocturnal haemoglobinuria. ( 27046923 )
2016
35
Challenging Airway Secondary to Purpura Fulminans With Face and Neck Bullae in a Premature Infant. ( 27941484 )
2016
36
Severe Neonatal Purpura Fulminans Caused by Staphylococcus aureus. ( 27449990 )
2016
37
Acute infectious purpura fulminans caused by group A I^-hemolytic Streptococcus: An uncommon organism. ( 27057503 )
2016
38
Delayed Surgical Debridement and Use of Semiocclusive Dressings for Salvage of Fingers After Purpura Fulminans. ( 28149224 )
2016
39
Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult. ( 27583208 )
2016
40
Efficacy and safety of protein C concentrate to treat purpura fulminans and thromboembolic events in severe congenital protein C deficiency. ( 27052576 )
2016
41
Pravastatin-induced rhabdomyolysis and purpura fulminans in a patient with chronic renal failure. ( 25644555 )
2015
42
Purpura fulminans 10 years after contaminated cocaine use. ( 26028121 )
2015
43
Bilateral symmetrical digital gangrene of upper and lower limbs due to purpura fulminans caused by Streptococcus pyogenes: A rare entity. ( 25983440 )
2015
44
Purpura Fulminans due to Enterobacter cloacae. ( 26370876 )
2015
45
Infectious purpura fulminans. ( 25857509 )
2015
46
Angioimmunoblastic t-cell lymphoma presenting as purpura fulminans. ( 25750965 )
2015
47
Purpura fulminans in the setting of Escherichia coli septicemia. ( 26761941 )
2015
48
Purpura Fulminans Due to Acquired Protein C Deficiency. ( 26677306 )
2015
49
Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia-A Tertiary Center's Experience. ( 26436558 )
2015
50
Purpura Fulminans Secondary to Rickettsial Infection: A Case Report. ( 26673422 )
2015

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
12.78 F2 F3 PROC PROCR PROS1 SERPINC1
2
Show member pathways
12.44 F2 F3 PROC PROS1 SERPINC1
3
Show member pathways
11.92 F2 PROC PROCR PROS1
4
Show member pathways
11.7 F2 F3 PROC PROCR PROS1 SERPINC1
5
Show member pathways
11.61 F2 PROC PROS1
6 11.31 F2 F3 PROC PROCR PROS1 SERPINC1
7 10.61 F2 PROC PROS1

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 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 9.67 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 regulation of blood coagulation GO:0030193 9.51 F2 SERPINC1
8 fibrinolysis GO:0042730 9.5 F2 PROS1 SERPINF2
9 negative regulation of blood coagulation GO:0030195 9.48 PROC PROS1
10 negative regulation of fibrinolysis GO:0051918 9.43 F2 SERPINF2
11 signal peptide processing GO:0006465 9.43 F2 PROC PROS1
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 for Purpura Fulminans

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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