SS
MCID: ACT100
MIFTS: 56

Acute Febrile Neutrophilic Dermatosis (SS) malady

Rare diseases, Skin diseases categories
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Summaries for Acute Febrile Neutrophilic Dermatosis

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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 leukemia and vasculitis, and has symptoms including facial pain/cephalalgia/migraine, thrombocytopenia/thrombopenia and hematuria/microhematuria. An important gene associated with Acute Febrile Neutrophilic Dermatosis is CTSG (cathepsin G), and among its related pathways are Matrix Metalloproteinases and Selected targets of C EBPalpha. The compounds elastatinal and 4-nitroanilide have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and bone, and related mouse phenotypes are immune system and hematopoietic system.

Description from OMIM:47 608068

Aliases & Classifications for Acute Febrile Neutrophilic Dermatosis

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43NIH Rare Diseases, 45Novoseek, 49Orphanet, 47OMIM, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 49 
Rare skin diseases


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 Orphanet59 84625002
UMLS via Orphanet63 C0085077

Related Diseases for Acute Febrile Neutrophilic Dermatosis

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Sources:
17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Acute Febrile Neutrophilic Dermatosis:



Diseases related to acute febrile neutrophilic dermatosis

Symptoms for Acute Febrile Neutrophilic Dermatosis

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Sources:
47OMIM, 49Orphanet
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Clinical features from OMIM:

608068

Symptoms:

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

Drugs & Therapeutics for Acute Febrile Neutrophilic Dermatosis

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Acute Febrile Neutrophilic Dermatosis

Search NIH Clinical Center for Acute Febrile Neutrophilic Dermatosis

Genetic Tests for Acute Febrile Neutrophilic Dermatosis

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Anatomical Context for Acute Febrile Neutrophilic Dermatosis

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

33
Skin, Liver, Bone, Lung, Prostate, Heart, Breast, Myeloid

Animal Models for Acute Febrile Neutrophilic Dermatosis or affiliated genes

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Sources:
37MGI
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MGI Mouse Phenotypes related to Acute Febrile Neutrophilic Dermatosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.1CTSG, CSF3, ELANE, PIK3C2A
2MP:00053977.8CTSG, CSF3, ELANE, PIK3C2A

Publications for Acute Febrile Neutrophilic Dermatosis

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Sources:
52PubMed
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Articles related to Acute Febrile Neutrophilic Dermatosis:

(show top 50)    (show all 111)
idTitleAuthorsYear
1
Azathioprine-induced acute febrile neutrophilic dermatosis (Sweet's syndrome). (23955987)
2013
2
Acute febrile neutrophilic dermatosis (Sweet's syndrome). (23207661)
2013
3
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a child, associated with a rotavirus infection: a case report. (20727148)
2010
4
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases. (18830624)
2009
5
Acute febrile neutrophilic dermatosis (Sweet's syndrome): a study of 15 cases in Iran. (17550553)
2007
6
Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. (17414432)
2007
7
From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research. (17097401)
2006
8
Subcutaneous Sweet's syndrome: a variant of acute febrile neutrophilic dermatosis that is included in the histopathologic differential diagnosis of neutrophilic panniculitis. (15858502)
2005
9
What is your diagnosis? Sweet Syndrome (acute febrile neutrophilic dermatosis). (14655771)
2003
10
Heart failure in acute febrile neutrophilic dermatosis. (14585639)
2003
11
Acute febrile neutrophilic dermatosis (Sweet's syndrome) with nodular episcleritis and polyneuropathy. (11982648)
2002
12
Acute febrile neutrophilic dermatosis following tuberculous infection. (12568224)
2002
13
Sweet's syndrome (acute febrile neutrophilic dermatosis). (10673451)
1999
14
Scleritis associated with acute febrile neutrophilic dermatosis (Sweet's syndrome) (9602638)
1998
15
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with relapsing polychondritis. (9892977)
1998
16
Acute febrile neutrophilic dermatosis (Sweet's syndrome) of the head and neck. (9664250)
1998
17
Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome. (8621829)
1996
18
Multiple erythematous tender papules and nodules in an 11-month-old boy. Sweet syndrome (SS) (acute febrile neutrophilic dermatosis). (8961884)
1996
19
Sweet syndrome: acute febrile neutrophilic dermatosis. (7640199)
1995
20
Sweet's syndrome (acute febrile neutrophilic dermatosis) (8089280)
1994
21
Acute febrile neutrophilic dermatosis (Sweet's syndrome). (8486457)
1993
22
Acute febrile neutrophilic dermatosis in association with erythema nodosum and sarcoidosis. (8440052)
1993
23
Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with BehAset's disease. (1459764)
1992
24
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in metastatic breast cancer. (1515281)
1992
25
Acute bullous hemorrhagic eruption. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (2181942)
1990
26
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
27
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Association with rheumatoid vasculitis. (2757408)
1989
28
Fever and painful plaques on the face, back, and extremities. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (2673050)
1989
29
Acute febrile neutrophilic dermatosis (Sweet's syndrome). A report of 2 cases. (2928884)
1989
30
Acute febrile neutrophilic dermatosis (Sweet's syndrome) and adenocarcinoma of the rectum. (3214949)
1988
31
Pyoderma gangrenosum versus acute febrile neutrophilic dermatosis (Sweet's syndrome) (3674086)
1987
32
Rapid onset of pustular plaques. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (3827286)
1987
33
Pulmonary involvement in Sweet's syndrome (acute febrile neutrophilic dermatosis). Preleukemic and leukemic phases of acute myelogenous leukemia. (3465510)
1986
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) and the related conditions of &quot;bowel bypass&quot; syndrome and bullous pyoderma gangrenosum. (3912087)
1985
36
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Successful treatment with short term corticosteroids. (4087247)
1985
37
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Response to dapsone. (6696481)
1984
38
Colchicine in the treatment of acute febrile neutrophilic dermatosis (Sweet's syndrome). (6821647)
1983
39
Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders. (6572088)
1983
40
Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis. (6822929)
1983
41
Sweet's syndrome (acute febrile neutrophilic dermatosis): report of two cases. (6841165)
1983
42
Sweet's syndrome (acute febrile neutrophilic dermatosis). (6754235)
1982
43
Acute febrile neutrophilic dermatosis. Sweet's syndrome. (7283460)
1981
44
Acute febrile neutrophilic dermatosis (Sweet syndrome). (1239237)
1975
45
Sweet's syndrome. A further case of acute febrile neutrophilic dermatosis. (4615954)
1974
46
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Report of two cases and review of the literature. (4100335)
1971
47
Sweet's syndrome (acute febrile neutrophilic dermatosis). Report of a case. (5539508)
1971
48
Acute febrile neutrophilic dermatosis (Sweet). (5374320)
1969
49
Further observations on acute febrile neutrophilic dermatosis. (5706798)
1968
50
AN ACUTE FEBRILE NEUTROPHILIC DERMATOSIS. (14201182)
1964

Variations for Acute Febrile Neutrophilic Dermatosis

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Expression for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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2BioGPS, 15Gene Expression Omnibus DataSets
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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

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Sources:
50PathCards, 38NCBI BioSystems Database, 53QIAGEN, 55Reactome, 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
1
Show member pathways
9.4CTSG, ELANE
29.4CTSG, ELANE

Compounds for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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Sources:
45Novoseek, 3BitterDB, 11DrugBank, 29IUPHAR, 24HMDB, 61Tocris Bioscience, 51PharmGKB
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Compounds related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

(show top 50)    (show all 58)
idCompoundScoreTop Affiliating Genes
1elastatinal459.8ELANE, CTSG
24-nitroanilide459.8ELANE, CTSG
3saccharin45 310.8CTSG, ELANE
4(4-aminophenyl)mercuric acetate459.8ELANE, CTSG
5thermolysin459.8CTSG, ELANE
6pmsf459.8ELANE, CTSG
7hocl459.8ELANE, CTSG
8contrapsin459.8ELANE, SERPINA3
9epsilon aminocaproic acid459.8CTSG, ELANE
10pegfilgrastim45 1110.8CSF3, ELANE
11phosphoramidon45 1110.7ELANE, CTSG
12propylthiouracil45 3 1111.7ELANE, CSF3
13filgrastim45 1110.7CSF3, ELANE
14cytochalasin b459.7ELANE, CTSG
15gm-csf45 2910.7CSF3, ELANE
16kininogen459.7CTSG, ELANE
17aprotinin45 1110.6CTSG, ELANE
18phosphocreatine45 2410.5PIK3C2A, CTSG
19leukotriene b445 61 2411.5ELANE, CTSG
20sodium dodecylsulfate459.5CTSG, ELANE
21xanthine45 2410.5CTSG, PIK3C2A
22malondialdehyde459.4PIK3C2A, CTSG
23thromboxane a245 2410.4CTSG, PIK3C2A
24melphalan45 51 1111.4CSF3, PIK3C2A
25nitroglycerin45 1110.3ELANE, PIK3C2A
26chymostatin459.3SERPINA3, ELANE, CTSG
27diisopropylfluorophosphate459.3CTSG, ELANE, SERPINA3
28n-formylmethionyl-leucylphenylalanine459.3CTSG, CSF3, ELANE
29endotoxin459.3CTSG, CSF3, ELANE
30valine459.2CSF3, ELANE, SERPINA3
31urea45 24 1111.1PIK3C2A, SERPINA3, CTSG
32sodium nitroprusside459.0PIK3C2A, ELANE
33prednisolone45 29 1111.0CSF3, PIK3C2A
34methotrexate51 45 1111.0CSF3, SERPINA3, PIK3C2A
35prostacyclin459.0PIK3C2A, ELANE, CTSG
36creatinine459.0CTSG, SERPINA3, PIK3C2A
37thyroxine45 249.9ELANE, SERPINA3, PIK3C2A
38lactate458.9PIK3C2A, ELANE, CTSG
39acetylcholine45 51 29 24 1112.9ELANE, SERPINA3, PIK3C2A
40arginine458.9CTSG, SERPINA3, PIK3C2A
41cyclosporin a45 29 6110.8CSF3, ELANE, PIK3C2A
42gold458.8SERPINA3, PIK3C2A
43fmlp458.8SERPINA3, ELANE, CSF3, CTSG
44retinoic acid45 249.7CTSG, CSF3, ELANE, SERPINA3
45alanine458.7CTSG, SERPINA3, PIK3C2A
46oxygen45 249.5ELANE, SERPINA3, PIK3C2A
47epinephrine45 24 1110.5PIK3C2A, ELANE, CSF3, CTSG
48fibrinogen458.5CTSG, ELANE, SERPINA3, PIK3C2A
49heparin45 29 24 1111.5PIK3C2A, SERPINA3, ELANE, CTSG
50superoxide45 249.0CTSG, CSF3, ELANE, SERPINA3, PIK3C2A

GO Terms for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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16Gene Ontology
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Cellular components related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.4CTSG, ELANE
2extracellular vesicular exosomeGO:0700627.8CTSG, ELANE, SERPINA3, PIK3C2A

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

idNameGO IDScoreTop Affiliating Genes
1positive regulation of immune responseGO:0507789.5CTSG, ELANE
2negative regulation of growth of symbiont in hostGO:0441309.4CTSG, ELANE
3proteolysisGO:0065089.4CTSG, ELANE
4extracellular matrix disassemblyGO:0226179.3ELANE, CTSG
5response to lipopolysaccharideGO:0324969.1CTSG, ELANE

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

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:0042529.4CTSG, ELANE
2peptidase activityGO:0082339.3CTSG, ELANE
3heparin bindingGO:0082019.1CTSG, ELANE

Products for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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  • Antibodies
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Sources for Acute Febrile Neutrophilic Dermatosis

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet