MCID: PRP034
MIFTS: 52

Purpura Fulminans malady

Blood diseases, Rare diseases categories

Summaries for Purpura Fulminans

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66Wikipedia, 34MalaCards
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Wikipedia:66 Purpura fulminans (also known as \"Purpura gangrenosa\") is an acute, often fatal, thrombotic disorder... more...

MalaCards: Purpura Fulminans is related to purpura 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 are Selected targets of HNF1 and PTM- gamma carboxylation, hypusine formation and arylsulfatase activation. The compounds certoparin and hirugen have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and brain, and related mouse phenotypes are embryogenesis and cardiovascular system.

Aliases & Classifications for Purpura Fulminans

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50Orphanet, 63UMLS, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 37MESH via Orphanet, 27ICD10 via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Blood diseases


Characteristics (Orphanet epidemiological data):

50
purpura fulminans:
Age of onset: Neonatal/infancy; Age of death: Before age 5


Aliases & Descriptions:

purpura fulminans 50 63


External Ids:

SNOMED-CT via Orphanet60 13507004
UMLS via Orphanet64 C0043068, C0085650
MESH via Orphanet37 D014884, D055665
ICD10 via Orphanet27 D65

Related Diseases for Purpura Fulminans

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18GeneCards, 19GeneDecks
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Diseases related to Purpura Fulminans via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 135)
idRelated DiseaseScoreTop Affiliating Genes
1purpura32.3SERPINC1
2protein s deficiency31.2SERPINC1, F5
3meningococcemia31.1SERPINC1, F5, PROCR
4sepsis31.1SERPINC1, F3, PROCR, PROC, LACTB, C1S
5protein c deficiency31.1PROCR, F2, APOH, SERPINC1, PROC, MTHFR
6disseminated intravascular coagulation30.8F2, SERPINC1, PROC, F3, PLAT, F5
7septic shock30.4F5, CSF3, F3, SERPINC1, LACTB, C1S
8lupus erythematosus30.4APOH, C1S, F2, F3, PROCR, SERPINC1
9systemic lupus erythematosus30.3F3, PROCR, C1S, F2, APOH
10endocarditis30.3LACTB
11toxic shock syndrome30.3LACTB
12hemolytic anemia30.2LACTB, F3, APOH
13endophthalmitis30.2LACTB, PLAT
14vasculitis30.2APOH, C1S, F2
15angioedema30.2C1S, PLAT
16peritonitis30.2LACTB, F2
17factor vii deficiency30.2F5, F3, SERPINC1, F2
18galactosemia30.2F2, F3, SERPINC1
19connective tissue disease30.2APOH, F3, F2, C1S
20pneumonia10.6
21chickenpox10.6
22meningitis10.6
23burns10.5
24malaria10.4
25necrotizing fasciitis10.4
26scarlet fever10.4
27fasciitis10.4
28influenza10.4
29arthritis10.3
30leukemia10.3
31branch retinal artery occlusion10.1F5
32limb ischemia10.1
33thrombotic thrombocytopenic purpura10.1
34polyarteritis nodosa10.1
35ulcerative colitis10.1
36measles10.1
37streptococcal meningitis10.1
38churg-strauss syndrome10.1
39osteonecrosis10.1
40hereditary angioedema10.1
41subdural empyema10.1
42mixed connective tissue disease10.1
43stevens-johnson syndrome10.1
44dengue hemorrhagic fever10.1
45cryptococcosis10.1
46aplastic anemia10.1
47chronic lymphocytic leukemia10.1
48conjunctivitis10.1
49hemoglobinopathy10.1
50hepatitis10.1

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



Diseases related to purpura fulminans

Symptoms for Purpura Fulminans

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Drugs & Therapeutics for Purpura Fulminans

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Purpura Fulminans

Drug clinical trials:

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Search NIH Clinical Center for Purpura Fulminans

Search CenterWatch for Purpura Fulminans

Genetic Tests for Purpura Fulminans

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Anatomical Context for Purpura Fulminans

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34MalaCards
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MalaCards organs/tissues related to Purpura Fulminans:

34
Skin, Bone, Brain, Liver, Breast

Animal Models for Purpura Fulminans or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Purpura Fulminans:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053808.7F5, F3, PROCR, SERPINC1, F2
2MP:00053858.1F2, SERPINC1, PROC, F3, PLAT, F5
3MP:00107718.0F2, F3, MTHFR, CSF3, F5
4MP:00053977.8F2, SERPINC1, PROC, PROCR, F3, PLAT
5MP:00053877.7F2, SERPINC1, PROC, PROCR, F3, PLAT
6MP:00036317.7F2, SERPINC1, PROC, F3, MTHFR, PLAT
7MP:00053787.7F2, PROC, PROCR, F3, MTHFR, PLAT
8MP:00053767.1F5, F2, APOH, SERPINC1, PROC, PROCR
9MP:00107687.1F2, APOH, SERPINC1, PROC, PROCR, F3

Publications for Purpura Fulminans

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Sources:
53PubMed
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Articles related to Purpura Fulminans:

(show top 50)    (show all 383)
idTitleAuthorsYear
1
A novel mutation c.1048A>T at codon 350(Lys>Stop) in PROC gene causing neonatal purpura fulminans. (24158118)
2013
2
Idiopathic purpura fulminans. (20674230)
2011
3
Diagnosis and management of neonatal purpura fulminans. (21839696)
2011
4
Purpura fulminans: recognition, diagnosis and management. (21233082)
2011
5
Human skin allografts as a useful adjunct in the treatment of purpura fulminans. (20852508)
2010
6
Purpura fulminans in a patient with rheumatoid arthritis. (19997707)
2009
7
Varicella-associated purpura fulminans and multiple deep vein thromboses: a case report. (19602824)
2009
8
Hypothesis: is the skin and tissue loss associated with septic purpura fulminans temperature related? Parallels with the anatomical distribution of frostbite. (18221840)
2008
9
Capnocytophaga ochracea causing severe sepsis and purpura fulminans in an immunocompetent patient. (16882454)
2007
10
Hereditary angioedema associated with heterozygous factor V Leiden mutation in a patient with Purpura fulminans. (17068406)
2007
11
Purpura fulminans: an unusual manifestation of severe falciparum malaria. (17588627)
2007
12
Suitable age for nasal reconstruction after subtotal amputation in a child, with respect to a case involving purpura fulminans. (16952604)
2006
13
Purpura fulminans during meningococcal sepsis treated with Drotrecogin alpha. A clinical case. (16570037)
2006
14
Purpura fulminans secondary to transient protein C deficiency as a complication of chickenpox infection. (16918648)
2006
15
Massive soft tissue infections: necrotizing fasciitis and purpura fulminans. (15715517)
2005
16
Adult purpura fulminans associated with staphylococcal infection and administration of colony-stimulating factors. (12609794)
2003
17
Homozygous protein C deficiency with purpura fulminans: report of a new case and a description of a novel mutation. (12695756)
2003
18
A case report of purpura fulminans secondary to transient protein C deficiency as a complication of chickenpox infection. (12718382)
2003
19
An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia. (11090052)
2000
20
Postinfection purpura fulminans in a patient heterozygous for prothrombin G20210A and acquired protein S resistance. (10599098)
1999
21
Reconstructive surgery in children after meningococcal purpura fulminans. (10235331)
1999
22
Protein-C concentrate for meningococcal purpura fulminans. (9734967)
1998
23
Acute infectious purpura fulminans: pathogenesis and medical management. (9655311)
1998
24
Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans. (9393338)
1997
25
Neonatal purpura fulminans in association with factor V R506Q mutation. (8627449)
1996
26
Management strategy in purpura fulminans with multiple organ failure in children. (8719318)
1996
27
Treatment of purpura fulminans in meningococcemia with protein C concentrate. (7699550)
1995
28
Meningococcal purpura fulminans treated with antithrombin III concentrate: what is the optimal replacement therapy? (7848164)
1994
29
Epidural sympathetic blockade to relieve vascular insufficiency in an infant with purpura fulminans. (8375170)
1993
30
Epidemic meningococcemia and purpura fulminans with induced protein C deficiency. (8399877)
1993
31
Purpura fulminans due to protein S deficiency following chickenpox. (1386247)
1992
32
Streptococcal gangrene and purpura fulminans. (1298264)
1992
33
Successful treatment of neonatal purpura fulminans with epoprostenol. (1744852)
1991
34
Purpura fulminans and adrenal hemorrhage due to group Y meningococcemia in an elderly woman. (1901668)
1991
35
Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate. (1944440)
1991
36
More questions about purpura fulminans. (2297246)
1990
37
Acquired purpura fulminans. (2281320)
1990
38
Anticoagulant combination therapy of gabexate mesilate and urokinase in purpura fulminans. (3144146)
1987
39
Purpura fulminans following late-onset group B beta-hemolytic streptococcal sepsis. (6383017)
1984
40
Amputation following meningococcemia. A sequela to purpura fulminans. (6705382)
1984
41
Purpura fulminans. (6461269)
1982
42
Dilantin-induced disseminated intravascular coagulation with purpura fulminans. A case report. (1147459)
1975
43
Fatal purpura fulminans following total cardiopulmonary bypass. (4767451)
1973
44
Purpura fulminans. (4936527)
1971
45
Purpura fulminans treated with hyperbaric oxygen. (5475825)
1970
46
Microangiopathic hemolytic anemia caused by purpura fulminans. (5653626)
1968
47
Purpura fulminans associated with septicaemia. (6080580)
1967
48
PURPURA FULMINANS. REPORT OF A CASE SUCCESSFULLY TREATED WITH HEPARIN AND HYDROCORTISONE. REVIEW OF 50 CASES FROM THE LITERATURE. (14214897)
1964
49
Purpura fulminans; report of a case coincident with varicella. (13199925)
1954
50
PURPURA FULMINANS FOLLOWING A MILD CASE OF SCARLET FEVER. (20774576)
1929

Variations for Purpura Fulminans

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Expression for genes affiliated with Purpura Fulminans

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Purpura Fulminans

Search GEO for disease gene expression data for Purpura Fulminans.

Pathways for genes affiliated with Purpura Fulminans

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Sources:
51PathCards, 13EMD Millipore, 56Reactome, 52PharmGKB, 39NCBI BioSystems Database, 54QIAGEN, 55R&D Systems, 31KEGG
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Pathways related to Purpura Fulminans according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8PROC, APOH
2
Show member pathways
9.8PROC, F2
3
Show member pathways
thioredoxin pathway39
8.9F2, MTHFR, PLAT
4
Show member pathways
8.8F2, SERPINC1, PROC, F3, F5
5
Show member pathways
8.2F2, SERPINC1, PROC, F3, PLAT, F5
6
Show member pathways
8.2F2, SERPINC1, PROC, F3, PLAT, F5
7
Show member pathways
Complement Activation, Classical Pathway39
Complement and Coagulation Cascades39
7.8F5, F2, C1S, SERPINC1, PROC, F3

Compounds for genes affiliated with Purpura Fulminans

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Sources:
46Novoseek, 12DrugBank, 3BitterDB, 52PharmGKB, 25HMDB, 62Tocris Bioscience, 30IUPHAR
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Compounds related to Purpura Fulminans according to GeneCards/GeneDecks:

(show top 50)    (show all 152)
idCompoundScoreTop Affiliating Genes
1certoparin469.6SERPINC1, F2, F3
2hirugen469.5F5, F2, SERPINC1
3inogatran469.5F3, F5, F2, SERPINC1
4ecarin469.4F5, F3, SERPINC1, F2
5danaparoid469.4F2, SERPINC1, F3, F5
6reviparin469.4SERPINC1, F3, PLAT
7ximelagatran46 1210.4F5, F2, SERPINC1, F3
8heparinoids469.4F3, SERPINC1, F2, F5
9efegatran469.4PLAT, F3, SERPINC1
10kaolin469.3F2, APOH, F5, F3
11coumarins469.3F2, APOH, F5, F3
12heparin sodium469.2PLAT, F2, F3
13coumarin46 3 52 2512.2F5, F3, F2, SERPINC1
14gamma-carboxyglutamic acid469.2F2, PROC, PROCR, F3, F5
15dextran 70469.2PLAT, F2, F3
16ancrod469.2SERPINC1, F5, PLAT, F2
17hydroxyethyl starch469.1F3, SERPINC1, F2, CSF3
18ticlopidine46 1210.0PLAT, F3, SERPINC1, APOH
19polybrene469.0SERPINC1, F3, F2
20ppack469.0SERPINC1, F2, F3, PLAT
21tirofiban46 1210.0F2, PLAT, F3, SERPINC1
22epsilon aminocaproic acid469.0F2, SERPINC1, F3, PLAT
23cardiolipin46 1210.0F5, F3, APOH, F2
24spectrozyme468.9SERPINC1, F3, PLAT, F5, F2
25fondaparinux468.9F2, SERPINC1, F3, PLAT, F5
26bivalirudin46 129.8PLAT, F3, SERPINC1, F2, F5
27argatroban46 129.8PLAT, F2, SERPINC1, F3, F5
28protamine sulfate468.8F2, SERPINC1, F3, PLAT, F5
29ristocetin468.8F5, F2, SERPINC1, PLAT, F3
30levonorgestrel46 62 30 1211.8PLAT, F3, SERPINC1, F2, F5
31desmopressin46 62 30 1211.8SERPINC1, PLAT, F2, F3, F5
32tranexamic acid46 129.8PLAT, F2, C1S, SERPINC1, F3
33protamine468.8F5, PLAT, F3, F2, SERPINC1
34dermatan sulfate468.8F5, PLAT, F3, SERPINC1, F2
35clopidogrel46 52 25 1211.8PLAT, SERPINC1, F2, F3
36abciximab46 129.8SERPINC1, C1S, F2, PLAT, F3
37phospholipid468.6F5, F3, PROC, PROCR, F2, APOH
38endotoxin468.6PROCR, APOH, F5, F3, CSF3
39hirudin468.6PLAT, F2, SERPINC1, F3, F5, PROCR
40aprotinin46 129.5C1S, F2, SERPINC1, F3, PLAT, F5
41prostacyclin468.5F5, PLAT, F3, SERPINC1, APOH, C1S
42cyclophosphamide46 52 1210.3F5, CSF3, MTHFR, F3, APOH
43kininogen468.1APOH, F2, C1S, SERPINC1, F3, PLAT
44homocysteine46 258.9APOH, F3, SERPINC1, MTHFR, F5, F2
45creatinine467.8F5, PLAT, MTHFR, F2, SERPINC1, F3
46warfarin46 52 25 1210.7F2, PLAT, F5, F3, PROC, SERPINC1
47aspirin46 52 30 2510.6F5, MTHFR, SERPINC1, APOH, C1S, PLAT
48heparin46 30 25 1210.3SERPINC1, PLAT, MTHFR, F3, APOH, F2
49fibrinogen467.0SERPINC1, PLAT, PROC, MTHFR, PROCR, C1S
50serine466.5F2, C1S, F3, PLAT, LACTB, MTHFR

GO Terms for genes affiliated with Purpura Fulminans

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17Gene Ontology
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Cellular components related to Purpura Fulminans according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular matrixGO:0310129.1APOH, F3, PLAT
2blood microparticleGO:0725629.1C1S, SERPINC1, F2
3extracellular regionGO:0055768.1F5, F2, C1S, SERPINC1, PROC, PLAT
4extracellular spaceGO:0056157.5F3, F2, APOH, SERPINC1, PLAT, F5

Biological processes related to Purpura Fulminans according to GeneCards/GeneDecks:

(show all 11)
idNameGO IDScoreTop Affiliating Genes
1peptidyl-glutamic acid carboxylationGO:0171879.9PROC, F2
2negative regulation of blood coagulationGO:0301959.9PROC, APOH
3negative regulation of fibrinolysisGO:0519189.8APOH, F2
4positive regulation of blood coagulationGO:0301949.8F2, APOH
5blood circulationGO:0080159.7F5, MTHFR
6plasminogen activationGO:0316399.7APOH, PLAT
7blood coagulation, intrinsic pathwayGO:0075979.5F2, APOH
8negative regulation of proteolysisGO:0458619.5F2, PLAT
9fibrinolysisGO:0427309.4F2, PLAT
10proteolysisGO:0065088.8PLAT, PROC, F2
11blood coagulationGO:0075968.1F5, PLAT, F3, PROCR, PROC, SERPINC1

Molecular functions related to Purpura Fulminans according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:0042528.2F5, PLAT, PROC, C1S, F2
2protein bindingGO:0055157.1C1S, APOH, SERPINC1, PROC, PROCR, F3

Products for genes affiliated with Purpura Fulminans

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  • Antibodies
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Sources for Purpura Fulminans

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet