MCID: PRP034
MIFTS: 41

Purpura Fulminans malady

Categories: Blood diseases, Rare diseases

Aliases & Classifications for Purpura Fulminans

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 lupus erythematosus and influenza. 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 Fibrinolytic Agents and Anticoagulants have been mentioned in the context of this disorder. Affiliated tissues include skin, t cells and bone, and related phenotypes are cardiovascular system and hematopoietic system

Wikipedia : 71 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 182)
id Related Disease Score Top Affiliating Genes
1 lupus erythematosus 29.9 F3 SERPINC1
2 influenza 29.3 F2 F3 SERPINC1 SERPINF2
3 acquired purpura fulminans 12.2
4 thrombophilia due to protein c deficiency, autosomal recessive 11.2
5 thrombophilia due to protein s deficiency, autosomal recessive 11.2
6 purpura 11.0
7 protein c deficiency 10.4
8 protein s deficiency 10.3
9 pneumonia 10.2
10 meningococcemia 10.2
11 disseminated intravascular coagulation 10.2
12 hemarthrosis 10.2 F2 F3
13 central congenital hypothyroidism 10.2 F3 PROC
14 alpha-2-macroglobulin deficiency 10.2 F2 F3
15 hendra virus infection 10.2 F2 F3
16 prostatic hypertrophy 10.2 F2 F3
17 dyskinetic cerebral palsy 10.2 F2 F3
18 stroke, ischemic 10.2 F2 F3
19 sporotrichosis 10.2 F2 F3
20 chronic inflammatory demyelinating polyneuritis 10.2 F2 F3
21 seminoma 10.2 F2 F3
22 brain stem cancer 10.1 F2 F3
23 fletcher factor deficiency 10.1 F2 F3
24 myotonic dystrophy 10.1 F2 F3
25 meningitis 10.1
26 vascular erectile tumor 10.1 F2 SERPINC1
27 spinocerebellar ataxia 27 10.1 F2 F3
28 may-hegglin anomaly 10.1 F2 SERPINC1
29 mobitz type ii atrioventricular block 10.1 F2 F3
30 retinal arterial macroaneurysm with supravalvular pulmonic stenosis 10.1 F2 F3
31 left-right axis malformations 10.1 F2 SERPINC1
32 lymphocytic colitis 10.1 F2 SERPINC1
33 schwannoma of jugular foramen 10.1 F2 SERPINC1
34 artery disease 10.1 F2 F3
35 bullous skin disease 10.1 F2 SERPINC1
36 red color blindness 10.1 F2 F3
37 neuroaxonal dystrophy 10.1 F2 SERPINC1
38 cerebellar astrocytoma 10.1 F2 SERPINC1
39 periosteal chondrosarcoma 10.1 F2 SERPINC1
40 chickenpox 10.1
41 angelucci's syndrome 10.1 F2 SERPINC1
42 sertoli cell tumor 10.1 F3 SERPINC1
43 hereditary endotheliopathy, retinopathy, nephropathy, and stroke 10.1 F3 SERPINC1
44 low anorectal malformation 10.1 F2 F3
45 hypertrichosis 10.1 F3 SERPINC1
46 cryptosporidiosis 10.1 F2 SERPINC1
47 angular blepharoconjunctivitis 10.1 F2 SERPINC1
48 otopalatodigital syndrome, type i 10.1 F2 F3
49 ovarian papillary cystadenoma 10.1 F2 SERPINC1
50 eagle syndrome 10.1 F2 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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.65 PROCR SERPINC1 F2 F3 PROC
2 hematopoietic system MP:0005397 9.55 PROCR SERPINC1 F2 F3 PROC
3 homeostasis/metabolism MP:0005376 9.43 PROCR SERPINC1 SERPINF2 F2 F3 PROC
4 immune system MP:0005387 9.02 PROC PROCR SERPINC1 F2 F3

Drugs & Therapeutics for Purpura Fulminans

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

(show all 10)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Fibrinolytic Agents Phase 2, Phase 3
2 Anticoagulants Phase 2, Phase 3
3 Protein C Phase 2, Phase 3
4
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754 657311
5
Butyric Acid Experimental Phase 2 107-92-6 264
6 Cortisol succinate Phase 2
7 Hydrocortisone 17-butyrate 21-propionate Phase 2
8 Hydrocortisone acetate Phase 2
9 Hydrocortisone-17-butyrate Phase 2
10 Anti-Inflammatory Agents Phase 2

Interventional clinical trials:


id Name Status NCT ID Phase
1 Efficacy and Safety Study of Protein C Concentrate in Subjects With Severe Congenital Protein C Deficiency Completed NCT00157118 Phase 2, Phase 3
2 Evaluation of Corticosteroid Therapy in Childhood Severe Sepsis - a Randomised Pilot Study Completed NCT00732277 Phase 2
3 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:

39
Skin, T Cells, Bone, Liver, Breast, Brain

Publications for Purpura Fulminans

Articles related to Purpura Fulminans:

(show top 50) (show all 416)
id Title Authors Year
1
Purpura Fulminans Associated with Overwhelming Postsplenectomy Infection. ( 28090064 )
2017
2
Purpura fulminans secondary to rickettsial infections: A case series. ( 26955583 )
2016
3
Acute infectious purpura fulminans caused by group A I^-hemolytic Streptococcus: An uncommon organism. ( 27057503 )
2016
4
Purpura fulminans associated with acute West Nile virus encephalitis. ( 26686320 )
2016
5
Purpura fulminans related to paroxysmal nocturnal haemoglobinuria. ( 27046923 )
2016
6
Fatal purpura fulminans and Waterhouse-Friderichsen syndrome from fulminant Streptococcus pneumoniae sepsis in an asplenic young adult. ( 27583208 )
2016
7
A Novel Protein C Mutation Causing Neonatal Purpura Fulminans. ( 27889735 )
2016
8
Strongyloides stercoralis hyperinfection in patient with autoimmune hepatitis and purpura fulminans. ( 26955218 )
2016
9
Successful Corticosteroid Treatment for Purpura Fulminans Associated with Quinolone. ( 27746448 )
2016
10
Streptococcus pneumoniae-induced purpura fulminans. ( 27083982 )
2016
11
Efficacy and safety of protein C concentrate to treat purpura fulminans and thromboembolic events in severe congenital protein C deficiency. ( 27052576 )
2016
12
Lifetime cost of meningococcal disease in France: Scenarios of severe meningitis and septicemia with purpura fulminans. ( 26688376 )
2016
13
Purpura fulminans and severe sepsis due to Pasteurella multocida infection in an immunocompetent patient. ( 27298290 )
2016
14
Pneumococcal Sepsis Complicated by Splenic Abscesses and Purpura Fulminans in a 15-Month-Old Child: Case Report and Review of the Literature. ( 27006958 )
2016
15
Challenging Airway Secondary to Purpura Fulminans With Face and Neck Bullae in a Premature Infant. ( 27941484 )
2016
16
Severe Neonatal Purpura Fulminans Caused by Staphylococcus aureus. ( 27449990 )
2016
17
Purpura fulminans in the setting of Escherichia coli septicemia. ( 26761941 )
2015
18
Pravastatin-induced rhabdomyolysis and purpura fulminans in a patient with chronic renal failure. ( 25644555 )
2015
19
Purpura fulminans 10 years after contaminated cocaine use. ( 26028121 )
2015
20
Angioimmunoblastic t-cell lymphoma presenting as purpura fulminans. ( 25750965 )
2015
21
Infectious purpura fulminans. ( 25857509 )
2015
22
Neonatal purpura fulminans due to protein C deficiency. ( 25783192 )
2015
23
Purpura Fulminans due to Enterobacter cloacae. ( 26370876 )
2015
24
Bilateral symmetrical digital gangrene of upper and lower limbs due to purpura fulminans caused by Streptococcus pyogenes: A rare entity. ( 25983440 )
2015
25
1102: PROTEIN C FOR TREATMENT OF SEPSIS ASSOCIATED PURPURA FULMINANS. ( 26570763 )
2015
26
Purpura Fulminans Due to Acquired Protein C Deficiency. ( 26677306 )
2015
27
Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia-A Tertiary Center's Experience. ( 26436558 )
2015
28
Purpura Fulminans Secondary to Rickettsial Infection: A Case Report. ( 26673422 )
2015
29
From the toe to the head: metastatic endophalmitis following staphylococcal purpura fulminans. ( 24231686 )
2014
30
Identification of differentially expressed serum proteins in infectious purpura fulminans. ( 24659849 )
2014
31
Indian tick typhus presenting as Purpura fulminans. ( 25097365 )
2014
32
Fatal purpura fulminans and septic shock in asplenic patient with Streptococcus pneumoniae bacteremia. ( 24407746 )
2014
33
Antiphospholipid antibody syndrome presenting with purpura fulminans. ( 23869929 )
2013
34
Pneumococcal sepsis-induced purpura fulminans in an asplenic adult patient without disseminated intravascular coagulation. ( 24185261 )
2013
35
Purpura fulminans: an unusual complication of malaria. ( 24076110 )
2013
36
Purpura Fulminans following Thermal Injury. ( 23762660 )
2013
37
Primary meningococcal arthritis leading to Neisseria meningitidies purpura fulminans. ( 23599862 )
2013
38
A novel mutation c.1048A>T at codon 350(Lys>Stop) in PROC gene causing neonatal purpura fulminans. ( 24158118 )
2013
39
Purpura fulminans in a patient with paroxysmal nocturnal haemoglobinuria. ( 23324094 )
2013
40
Plastic surgical treatment of purpura fulminans: Long-term follow-up of two patients. ( 23210495 )
2013
41
Purpura fulminans in an acute preterm neonate. ( 23250966 )
2013
42
Meningococcal interaction to microvasculature triggers the tissular lesions of purpura fulminans. ( 23840047 )
2013
43
Purpura fulminans caused by meningococcemia in an infant. ( 23921694 )
2013
44
Assessment of the interplay between blood and skin vascular abnormalities in adult purpura fulminans. ( 23924269 )
2013
45
Limb fitting for quadruple amputees: report of two cases of symmetrical peripheral gangrene caused by pneumococcal purpura fulminans. ( 23558402 )
2013
46
Purpura Fulminans after Therapeutic Hypothermia in an Asphyxiated Neonate with Streptococcemia. ( 23839905 )
2013
47
Purpura fulminans associated with Streptococcus pneumoniae septicemia in an asplenic pediatric patient. ( 23985086 )
2013
48
Streptococcus pneumoniae-induced purpura fulminans in a woman with functional asplenia. ( 23870635 )
2013
49
Purpura fulminans in a patient with mixed connective tissue disease. ( 23370948 )
2013
50
Neonatal purpura fulminans manifestation in early-onset group B Streptococcal infection. ( 23970945 )
2013

Variations for Purpura Fulminans

Expression for Purpura Fulminans

Search GEO for disease gene expression data for Purpura Fulminans.

Pathways for Purpura Fulminans

GO Terms for Purpura Fulminans

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

id Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.72 F2 F3 PROCR SERPINC1 SERPINF2
2 cell surface GO:0009986 9.54 F3 PROCR SERPINF2
3 endoplasmic reticulum lumen GO:0005788 9.43 F2 PROC SERPINC1
4 extracellular space GO:0005615 9.35 F2 F3 PROC SERPINC1 SERPINF2
5 blood microparticle GO:0072562 9.33 F2 SERPINC1 SERPINF2
6 extracellular region GO:0005576 9.1 F2 F3 PROC PROCR SERPINC1 SERPINF2

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

(show all 12)
id Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.65 F2 PROC SERPINC1
2 leukocyte migration GO:0050900 9.63 F2 PROC PROCR
3 acute-phase response GO:0006953 9.51 F2 SERPINF2
4 positive regulation of collagen biosynthetic process GO:0032967 9.49 F2 SERPINF2
5 regulation of blood coagulation GO:0030193 9.43 F2 SERPINC1
6 fibrinolysis GO:0042730 9.4 F2 SERPINF2
7 signal peptide processing GO:0006465 9.37 F2 PROC
8 blood coagulation GO:0007596 9.35 F2 F3 PROC PROCR SERPINC1
9 peptidyl-glutamic acid carboxylation GO:0017187 9.32 F2 PROC
10 negative regulation of fibrinolysis GO:0051918 9.26 F2 SERPINF2
11 negative regulation of coagulation GO:0050819 9.16 PROC PROCR
12 hemostasis GO:0007599 9.02 F2 F3 PROC PROCR SERPINC1

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

id Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase inhibitor activity GO:0004867 9.16 SERPINC1 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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