SS
MCID: ACT100
MIFTS: 49

Acute Febrile Neutrophilic Dermatosis (SS) malady

Skin category

Summaries for Acute Febrile Neutrophilic Dermatosis

Sources:
43NIH Rare Diseases, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 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 acute myocardial infarction, and has symptoms including dermal/subcutaneous infiltration/induration, storage liver disease and splenomegaly. 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, bone marrow and heart, and related mouse phenotype hematopoietic system.

Description from OMIM:47 608068

Aliases & Classifications for Acute Febrile Neutrophilic Dermatosis

Sources:
43NIH Rare Diseases, 45Novoseek, 49Orphanet, 47OMIM, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
sweet syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


Aliases & Descriptions:

acute febrile neutrophilic dermatosis 43 49
sweet syndrome 43 49 47
neutrophilic dermatosis, acute febrile 43
gomm button disease 43
sweets syndrome 45
ss 43


External Ids:

OMIM47 608068
MESH via Orphanet36 D016463
ICD10 via Orphanet26 L98.2
SNOMED-CT via Orphanet58 84625002
UMLS via Orphanet62 C0085077

Related Diseases for Acute Febrile Neutrophilic Dermatosis

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 151)
idRelated DiseaseScoreTop Affiliating Genes
1acute leukemia31.0CSF3
2acute myocardial infarction30.4PIK3C2A
3vasculitis30.4ELANE, CTSG
4arthritis30.4CTSG, SERPINA3
5sepsis30.4CTSG, CSF3, ELANE
6n syndrome11.3
7congenital heart block10.6
8adenocarcinoma10.6
9pyoderma gangrenosum10.5
10cutaneous lupus erythematosus10.5
11systemic lupus erythematosus10.5
12subacute cutaneous lupus erythematosus10.4
13hemoglobin d disease10.4
14hemoglobin c disease10.3
15relapsing polychondritis10.3
16facial dermatosis10.3
17cutis laxa10.3
18coccidioidomycosis10.3
19cellulitis10.3
20mixed connective tissue disease10.3
21dressler's syndrome10.3
22prostate transitional cell carcinoma10.3
23nodular episcleritis10.3
24conn's syndrome10.3
25anterior scleritis10.3
26bladder adenocarcinoma10.3
27bladder carcinoma10.3
28bladder transitional cell carcinoma10.3
29transitional cell carcinoma10.3
30short syndrome10.3
31chronic neutrophilic leukemia10.3
32essential thrombocythemia10.3
33myelodysplastic syndromes10.3
34rheumatoid vasculitis10.3
35back pain10.3
36myeloproliferative disorder10.3
37premature ejaculation10.2
38sickle cell disease10.2
39alpha thalassemia10.2
40thalassemia10.2
41sjogren's syndrome10.1
42synovial sarcoma10.1
43sickle cell - hemoglobin d disease10.1
44sickle cell - hemoglobin c disease10.1
45iritis10.0
46sickle cell anemia10.0
47hemochromatosis10.0
48neonatal hemochromatosis10.0
49hemoglobin ss10.0
50pituitary tumors10.0

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

Sources:
47OMIM, 49Orphanet
See all sources

Clinical features from OMIM:

608068

Symptoms:

49 (show all 33)
  • dermal/subcutaneous infiltration/induration
  • 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
  • macules
  • follicular/erythematous/edematous papules/milium
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • cough
  • myalgia/muscular pain
  • lung/pulmonary infiltrates
  • hematuria/microhematuria
  • thrombocytopenia/thrombopenia
  • 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

Drugs & Therapeutics for Acute Febrile Neutrophilic Dermatosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-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

Anatomical Context for Acute Febrile Neutrophilic Dermatosis

Sources:
33MalaCards
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MalaCards organs/tissues related to Acute Febrile Neutrophilic Dermatosis:

33
Skin, Bone marrow, Heart, Breast, Prostate, Myeloid

Animal Models for Acute Febrile Neutrophilic Dermatosis or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Acute Febrile Neutrophilic Dermatosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053978.1ELANE, PIK3C2A, CTSG, CSF3

Publications for Acute Febrile Neutrophilic Dermatosis

Sources:
51PubMed
See all sources

Articles related to Acute Febrile Neutrophilic Dermatosis:

(show top 50)    (show all 114)
idTitleAuthorsYear
1
Azathioprine-induced acute febrile neutrophilic dermatosis (Sweet's syndrome). (23955987)
2013
2
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with biliary sepsis. (24048149)
2013
3
MRI findings of neutrophilic fasciitis in a patient with acute febrile neutrophilic dermatosis (Sweet's syndrome). (21298430)
2011
4
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a child, associated with a rotavirus infection: a case report. (20727148)
2010
5
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases. (18830624)
2009
6
Ocular involvement in acute febrile neutrophilic dermatosis (Sweet syndrome): new cases and review of the literature. (18501268)
2008
7
Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. (17414432)
2007
8
Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with adenocarcinoma of prostate and transitional cell carcinoma of urinary bladder. (16164716)
2005
9
Heart failure in acute febrile neutrophilic dermatosis. (14585639)
2003
10
Aortic and mitral valve replacement in a patient with acute febrile neutrophilic dermatosis (Sweet's syndrome): report of a case. (11686561)
2001
11
A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) with palatal ulceration. (10760730)
2000
12
Scleritis associated with acute febrile neutrophilic dermatosis (Sweet's syndrome) (9602638)
1998
13
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with relapsing polychondritis. (9892977)
1998
14
Acute febrile neutrophilic dermatosis (Sweet's syndrome) of the head and neck. (9664250)
1998
15
Images in clinical medicine. Acute febrile neutrophilic dermatosis in acute myelogenous leukemia. (9834306)
1998
16
Concurrent Sweet's syndrome (acute febrile neutrophilic dermatosis), erythema nodosum and sarcoidosis. (9330058)
1997
17
Analysis of HLA antigens in Caucasian patients with acute febrile neutrophilic dermatosis (Sweet's syndrome). (9270521)
1997
18
Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome. (8621829)
1996
19
Multiple erythematous tender papules and nodules in an 11-month-old boy. Sweet syndrome (SS) (acute febrile neutrophilic dermatosis). (8961884)
1996
20
Sweet syndrome: acute febrile neutrophilic dermatosis. (7640199)
1995
21
Sweet's syndrome (acute febrile neutrophilic dermatosis) (8089280)
1994
22
Acute febrile neutrophilic dermatosis (Sweet's syndrome). (8486457)
1993
23
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in metastatic breast cancer. (1515281)
1992
24
Neutrophils in cerebrospinal fluid of a patient with acute febrile neutrophilic dermatosis (Sweet's syndrome) (1459771)
1992
25
Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with prostate adenocarcinoma and a myelodysplastic syndrome. (1459765)
1992
26
Acute bullous hemorrhagic eruption. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (2181942)
1990
27
Acute febrile neutrophilic dermatosis (Sweet syndrome). Case report and immunohistologic study of neutrophil-associated proteolytic enzymes and their inhibitors]. (2249945)
1990
28
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in association with myelodysplastic syndromes: a report of three cases and a review of the literature. (2679862)
1989
29
Fever and painful plaques on the face, back, and extremities. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (2673050)
1989
30
Acute febrile neutrophilic dermatosis. (2646883)
1989
31
Pyoderma gangrenosum versus acute febrile neutrophilic dermatosis (Sweet's syndrome) (3674086)
1987
32
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in BehAset's disease. (3593637)
1987
33
Acute febrile neutrophilic dermatosis (Sweet's syndrome) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia. (3469614)
1987
34
Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with post-myocardial infarction syndrome (Dressler's syndrome). (2411241)
1985
35
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Successful treatment with short term corticosteroids. (4087247)
1985
36
Acute febrile neutrophilic dermatosis (Sweet's syndrome): case report and review of the literature. (3906610)
1985
37
Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with lymphoma. (3988278)
1985
38
Relapsing acute febrile neutrophilic dermatosis and essential thrombocythemia. (6722359)
1984
39
A case of acute febrile neutrophilic dermatosis (Sweet's syndrome). A demonstration of IgM and C3 deposits on the vessel walls in involved skin. (6399056)
1984
40
Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders. (6572088)
1983
41
Acute febrile neutrophilic dermatosis--1978. (427014)
1979
42
Acute febrile neutrophilic dermatosis: Sweet's syndrome. (280960)
1978
43
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Four case reports. (653521)
1978
44
Acute febrile neutrophilic dermatosis associated with SjAPgren's syndrome. (677930)
1978
45
Acute febrile neutrophilic dermatosis (Sweet's syndrome) with myeloid proliferation. (687307)
1978
46
Sweet's syndrome. A further case of acute febrile neutrophilic dermatosis. (4615954)
1974
47
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Report of two cases and review of the literature. (4100335)
1971
48
Acute febrile neutrophilic dermatosis--two cases. (5131096)
1971
49
Acute febrile neutrophilic dermatosis. Sweet's syndrome. (5810898)
1969
50
Further observations on acute febrile neutrophilic dermatosis. (5706798)
1968

Genetic Variations for Acute Febrile Neutrophilic Dermatosis

Expression for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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

Sources:
12EMD Millipore
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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

Sources:
45Novoseek, 24HMDB, 2BitterDB, 11DrugBank, 60Tocris Bioscience, 50PharmGKB, 29IUPHAR
See all sources

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

(show all 43)
idCompoundScoreTop Affiliating Genes
1pmsf4510.4CTSG
24-nitroanilide459.8CTSG, ELANE
3elastatinal459.8CTSG, ELANE
4phosphocreatine45 2410.8CTSG, PIK3C2A
5saccharin45 210.8CTSG, ELANE
6(4-aminophenyl)mercuric acetate459.8ELANE, CTSG
7thermolysin459.8ELANE, CTSG
8hocl459.7CTSG, ELANE
9epsilon aminocaproic acid459.7CTSG, ELANE
10xanthine45 2410.7CTSG, PIK3C2A
11phosphoramidon45 1110.7CTSG, ELANE
12cytochalasin b459.7ELANE, CTSG
13malondialdehyde459.6CTSG, PIK3C2A
14nitroglycerin45 1110.6ELANE, PIK3C2A
15thromboxane a245 2410.6PIK3C2A, CTSG
16kininogen459.6ELANE, CTSG
17aprotinin45 1110.5ELANE, CTSG
18pegfilgrastim45 1110.4CSF3, ELANE
19leukotriene b445 60 2411.4ELANE, CTSG
20filgrastim45 1110.4ELANE, CSF3
21urea45 11 2411.4SERPINA3, PIK3C2A, CTSG
22melphalan45 1110.3PIK3C2A, CSF3
23chymostatin459.3CTSG, SERPINA3, ELANE
24diisopropylfluorophosphate459.3CTSG, SERPINA3, ELANE
25sodium dodecylsulfate459.3CTSG, ELANE
26prostacyclin459.3CTSG, PIK3C2A, ELANE
27creatinine459.2SERPINA3, PIK3C2A, CTSG
28thyroxine45 2410.2PIK3C2A, SERPINA3, ELANE
29lactate459.1CTSG, PIK3C2A, ELANE
30acetylcholine45 50 29 11 2413.1ELANE, SERPINA3, PIK3C2A
31sodium nitroprusside459.0ELANE, PIK3C2A
32n-formylmethionyl-leucylphenylalanine459.0CSF3, CTSG, ELANE
33methotrexate45 50 1111.0CSF3, PIK3C2A, SERPINA3
34arginine459.0SERPINA3, PIK3C2A, CTSG
35endotoxin459.0ELANE, CTSG, CSF3
36valine458.9ELANE, SERPINA3, CSF3
37cyclosporin a45 29 6010.8ELANE, PIK3C2A, CSF3
38fibrinogen458.8ELANE, SERPINA3, PIK3C2A, CTSG
39heparin45 29 11 2411.8ELANE, SERPINA3, PIK3C2A, CTSG
40fmlp458.5ELANE, SERPINA3, CTSG, CSF3
41epinephrine45 11 2410.5ELANE, PIK3C2A, CTSG, CSF3
42retinoic acid45 249.4ELANE, SERPINA3, CTSG, CSF3
43superoxide45 249.0ELANE, SERPINA3, PIK3C2A, CTSG, CSF3

GO Terms for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Acute Febrile Neutrophilic Dermatosis

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet