SS
MCID: ACT100
MIFTS: 43

Acute Febrile Neutrophilic Dermatosis (SS) malady

Skin diseases category

Summaries for Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
42NIH Rare Diseases, 46OMIM, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NIH Rare Diseases:42 Acute febrile neutrophilic dermatosis - also known as sweet syndrome - is a skin condition marked by fever, inflammation of the joints (arthritis), and painful skin lesions that appear mainly on the face, neck, back and arms. although middle-aged women are most likely to develop this condition, it may also affect men, older adults and even infants. the exact cause of acute febrile neutrophilic dermatosis often isn't known. in some people, it's triggered by an infection, illness or certain medications. this condition can also occur with some types of cancer and other serious health problems. most often, it isn't serious and will clear on its own in a few months. healing is much more rapid, however, with treatment. last updated: 7/23/2009

MalaCards: Acute Febrile Neutrophilic Dermatosis, also known as sweet syndrome, is related to acute leukemia and leukemia, and has symptoms including thrombocytopenia/thrombopenia, hematuria/microhematuria and lung/pulmonary infiltrates. An important gene associated with Acute Febrile Neutrophilic Dermatosis is CTSG (cathepsin G), and among its related pathways is Selected targets of C/EBPalpha. The compounds pmsf and 4-nitroanilide have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and liver, and related mouse phenotype hematopoietic system.

Description from OMIM:46 608068

Aliases & Classifications for Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
42NIH Rare Diseases, 48Orphanet, 46OMIM, 44Novoseek, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Skin diseases


Characteristics (Orphanet epidemiological data):

48
sweet syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


Aliases & Descriptions:

acute febrile neutrophilic dermatosis 42 48
sweet syndrome 42 48 46
neutrophilic dermatosis, acute febrile 42
gomm button disease 42
sweets syndrome 44
ss 42


External Ids:

OMIM46 608068
MESH via Orphanet35 D016463
ICD10 via Orphanet26 L98.2
SNOMED-CT via Orphanet57 84625002
UMLS via Orphanet61 C0085077

Related Diseases for Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Acute Febrile Neutrophilic Dermatosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 172)
idRelated DiseaseScoreTop Affiliating Genes
1acute leukemia31.0CSF3
2leukemia31.0SERPINA3, CTSG, CSF3
3sarcoidosis30.6SERPINA3
4acute myocardial infarction30.4PIK3C2A
5breast cancer30.4CSF3
6vasculitis30.4CTSG, ELANE
7myocardial infarction30.4PIK3C2A, SERPINA3
8arthritis30.4SERPINA3, CTSG
9rheumatoid arthritis30.0CTSG, SERPINA3, ELANE
10psoriasis29.8SERPINA3, ELANE
11pneumonia29.8ELANE, PIK3C2A, CTSG, CSF3
12malaria29.8SERPINA3
13melanoma29.7SERPINA3
14lupus erythematosus10.6
15adenocarcinoma10.5
16congenital heart block10.5
17pyoderma gangrenosum10.5
18hairy cell leukemia10.5
19prostate adenocarcinoma10.5
20prostatitis10.5
21pyoderma10.5
22scleritis10.5
23cutaneous lupus erythematosus10.4
24systemic lupus erythematosus10.4
25relapsing polychondritis10.3
26facial dermatosis10.3
27cutis laxa10.3
28coccidioidomycosis10.3
29cellulitis10.3
30mixed connective tissue disease10.3
31dressler's syndrome10.3
32prostate transitional cell carcinoma10.3
33nodular episcleritis10.3
34anterior scleritis10.3
35bladder adenocarcinoma10.3
36bladder carcinoma10.3
37bladder transitional cell carcinoma10.3
38connective tissue disease10.3
39fasciitis10.3
40influenza10.3
41multiple myeloma10.3
42myeloma10.3
43panniculitis10.3
44polyneuropathy10.3
45rectum adenocarcinoma10.3
46transitional cell carcinoma10.3
47premature ejaculation10.2
48sickle cell disease10.2
49sjogren's syndrome10.1
50sarcoma10.1

Graphical network of the top 20 diseases related to Acute Febrile Neutrophilic Dermatosis:



Diseases related to acute febrile neutrophilic dermatosis

Clinical Features for Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
46OMIM, 48Orphanet
See all sources

Clinical features from OMIM:

608068

Symptoms:

48 (show all 33)
  • thrombocytopenia/thrombopenia
  • hematuria/microhematuria
  • lung/pulmonary infiltrates
  • myalgia/muscular pain
  • cough
  • facial pain/cephalalgia/migraine
  • renal glomerular defect/glomerulopathy
  • biological inflammatory syndrome/increased erythrocyte sedimentation rate/crp
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • enanthema/aphtosa/aphta/leukoplakia
  • hyperleukocytosis/leukocytosis
  • pustula/pustulosis
  • red cell structure/shape anomalies
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • follicular/erythematous/edematous papules/milium
  • macules
  • storage liver disease
  • splenomegaly
  • repeat respiratory infections
  • malabsorption/chronic diarrhea/steatorrhea
  • anomalies of bones/skeletal anomalies
  • proteinuria
  • asthenia/fatigue/weakness
  • renal failure
  • fever/chilling
  • articular/joint pain/arthralgia
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • hyperkeratosis/ainhum/hyperkeratotic skin fissures
  • anaemia
  • chronic skin infection/ulcerations/ulcers/cancrum
  • cutaneous rash
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • dermal/subcutaneous infiltration/induration

Drugs & Therapeutics for Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Acute Febrile Neutrophilic Dermatosis

Drug clinical trials:

Search ClinicalTrials for Acute Febrile Neutrophilic Dermatosis

Search NIH Clinical Center for Acute Febrile Neutrophilic Dermatosis

Search CenterWatch for Acute Febrile Neutrophilic Dermatosis

Genetic Tests for Acute Febrile Neutrophilic Dermatosis

About this section

Anatomical Context for Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
32MalaCards
See all sources

MalaCards organs/tissues related to Acute Febrile Neutrophilic Dermatosis:

32
Skin, Lung, Liver, Bone

Animal Models for Acute Febrile Neutrophilic Dermatosis or affiliated genes

About this section
Sources:
36MGI
See all sources

MGI Mouse Phenotypes related to Acute Febrile Neutrophilic Dermatosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053978.1ELANE, PIK3C2A, CTSG, CSF3

Publications for Acute Febrile Neutrophilic Dermatosis

About this section

Genetic Variations for Acute Febrile Neutrophilic Dermatosis

About this section

Expression for genes affiliated with Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Acute Febrile Neutrophilic Dermatosis

Search GEO for disease gene expression data for Acute Febrile Neutrophilic Dermatosis.

Pathways for genes affiliated with Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
12EMD Millipore
See all sources

Pathways related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.4ELANE, CTSG

Compounds for genes affiliated with Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
44Novoseek, 24HMDB, 2BitterDB, 11DrugBank, 59Tocris Bioscience, 49PharmGKB, 28IUPHAR
See all sources

Compounds related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

(show all 43)
idCompoundScoreTop Affiliating Genes
1pmsf4410.4CTSG
24-nitroanilide449.8CTSG, ELANE
3elastatinal449.8CTSG, ELANE
4phosphocreatine44 2410.8CTSG, PIK3C2A
5saccharin44 210.8CTSG, ELANE
6(4-aminophenyl)mercuric acetate449.8ELANE, CTSG
7thermolysin449.8ELANE, CTSG
8hocl449.7CTSG, ELANE
9epsilon aminocaproic acid449.7CTSG, ELANE
10xanthine44 2410.7CTSG, PIK3C2A
11phosphoramidon44 1110.7CTSG, ELANE
12cytochalasin b449.7ELANE, CTSG
13malondialdehyde449.6CTSG, PIK3C2A
14nitroglycerin44 1110.6ELANE, PIK3C2A
15thromboxane a244 2410.6PIK3C2A, CTSG
16kininogen449.6ELANE, CTSG
17aprotinin44 1110.5ELANE, CTSG
18pegfilgrastim44 1110.4CSF3, ELANE
19leukotriene b444 59 2411.4ELANE, CTSG
20filgrastim44 1110.4ELANE, CSF3
21urea44 11 2411.4SERPINA3, PIK3C2A, CTSG
22melphalan44 1110.3PIK3C2A, CSF3
23chymostatin449.3CTSG, SERPINA3, ELANE
24diisopropylfluorophosphate449.3CTSG, SERPINA3, ELANE
25sodium dodecylsulfate449.3CTSG, ELANE
26prostacyclin449.3CTSG, PIK3C2A, ELANE
27creatinine449.2SERPINA3, PIK3C2A, CTSG
28thyroxine44 2410.2PIK3C2A, SERPINA3, ELANE
29lactate449.1CTSG, PIK3C2A, ELANE
30acetylcholine44 49 28 11 2413.1ELANE, SERPINA3, PIK3C2A
31sodium nitroprusside449.0ELANE, PIK3C2A
32n-formylmethionyl-leucylphenylalanine449.0CSF3, CTSG, ELANE
33methotrexate44 49 1111.0CSF3, PIK3C2A, SERPINA3
34arginine449.0SERPINA3, PIK3C2A, CTSG
35endotoxin449.0ELANE, CTSG, CSF3
36valine448.9ELANE, SERPINA3, CSF3
37cyclosporin a44 28 5910.8ELANE, PIK3C2A, CSF3
38fibrinogen448.8ELANE, SERPINA3, PIK3C2A, CTSG
39heparin44 28 11 2411.8ELANE, SERPINA3, PIK3C2A, CTSG
40fmlp448.5ELANE, SERPINA3, CTSG, CSF3
41epinephrine44 11 2410.5ELANE, PIK3C2A, CTSG, CSF3
42retinoic acid44 249.4ELANE, SERPINA3, CTSG, CSF3
43superoxide44 249.0ELANE, SERPINA3, PIK3C2A, CTSG, CSF3

GO Terms for genes affiliated with Acute Febrile Neutrophilic Dermatosis

About this section
Sources:
16Gene Ontology
See all sources

Cellular components related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.4ELANE, CTSG

Biological processes related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of immune responseGO:0507789.4ELANE, CTSG
2response to lipopolysaccharideGO:0324969.4ELANE, CTSG
3negative regulation of growth of symbiont in hostGO:0441309.3ELANE, CTSG
4extracellular matrix disassemblyGO:0226179.1ELANE, CTSG

Molecular functions related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1peptidase activityGO:0082339.4ELANE, CTSG

Products for genes affiliated with Acute Febrile Neutrophilic Dermatosis

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Acute Febrile Neutrophilic Dermatosis

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet