SS
MCID: ACT100
MIFTS: 56

Acute Febrile Neutrophilic Dermatosis (SS) malady

Rare diseases, Skin diseases categories

Summaries for Acute Febrile Neutrophilic Dermatosis

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Sources:
44NIH Rare Diseases, 48OMIM, 34MalaCards
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NIH Rare Diseases:44 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:48 608068

Aliases & Classifications for Acute Febrile Neutrophilic Dermatosis

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44NIH Rare Diseases, 46Novoseek, 50Orphanet, 48OMIM, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 50 
Rare skin diseases


Characteristics (Orphanet epidemiological data):

50
sweet syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


Aliases & Descriptions:

acute febrile neutrophilic dermatosis 44 50
sweet syndrome 44 50 48
neutrophilic dermatosis, acute febrile 44
gomm button disease 44
sweets syndrome 46
ss 44


External Ids:

OMIM48 608068
MESH via Orphanet37 D016463
ICD10 via Orphanet27 L98.2
SNOMED-CT via Orphanet60 84625002
UMLS via Orphanet64 C0085077

Related Diseases for Acute Febrile Neutrophilic Dermatosis

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18GeneCards, 19GeneDecks
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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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48OMIM, 50Orphanet
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Clinical features from OMIM:

608068

Symptoms:

50 (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:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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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

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Anatomical Context for Acute Febrile Neutrophilic Dermatosis

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34MalaCards
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MalaCards organs/tissues related to Acute Febrile Neutrophilic Dermatosis:

34
Skin, Liver, Bone, Lung, Prostate, Heart, Breast, Myeloid

Animal Models for Acute Febrile Neutrophilic Dermatosis or affiliated genes

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Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Acute Febrile Neutrophilic Dermatosis:

38
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:
53PubMed
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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, 16Gene 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:
51PathCards, 39NCBI BioSystems Database, 54QIAGEN, 56Reactome, 13EMD 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:
46Novoseek, 3BitterDB, 12DrugBank, 30IUPHAR, 25HMDB, 62Tocris Bioscience, 52PharmGKB
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Compounds related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

(show top 50)    (show all 58)
idCompoundScoreTop Affiliating Genes
1elastatinal469.8ELANE, CTSG
24-nitroanilide469.8ELANE, CTSG
3saccharin46 310.8CTSG, ELANE
4(4-aminophenyl)mercuric acetate469.8ELANE, CTSG
5thermolysin469.8CTSG, ELANE
6pmsf469.8ELANE, CTSG
7hocl469.8ELANE, CTSG
8contrapsin469.8ELANE, SERPINA3
9epsilon aminocaproic acid469.8CTSG, ELANE
10pegfilgrastim46 1210.8CSF3, ELANE
11phosphoramidon46 1210.7ELANE, CTSG
12propylthiouracil46 3 1211.7ELANE, CSF3
13filgrastim46 1210.7CSF3, ELANE
14cytochalasin b469.7ELANE, CTSG
15gm-csf46 3010.7CSF3, ELANE
16kininogen469.7CTSG, ELANE
17aprotinin46 1210.6CTSG, ELANE
18phosphocreatine46 2510.5PIK3C2A, CTSG
19leukotriene b446 62 2511.5ELANE, CTSG
20sodium dodecylsulfate469.5CTSG, ELANE
21xanthine46 2510.5CTSG, PIK3C2A
22malondialdehyde469.4PIK3C2A, CTSG
23thromboxane a246 2510.4CTSG, PIK3C2A
24melphalan46 52 1211.4CSF3, PIK3C2A
25nitroglycerin46 1210.3ELANE, PIK3C2A
26chymostatin469.3SERPINA3, ELANE, CTSG
27diisopropylfluorophosphate469.3CTSG, ELANE, SERPINA3
28n-formylmethionyl-leucylphenylalanine469.3CTSG, CSF3, ELANE
29endotoxin469.3CTSG, CSF3, ELANE
30valine469.2CSF3, ELANE, SERPINA3
31urea46 25 1211.1PIK3C2A, SERPINA3, CTSG
32sodium nitroprusside469.0PIK3C2A, ELANE
33prednisolone46 30 1211.0CSF3, PIK3C2A
34methotrexate52 46 1211.0CSF3, SERPINA3, PIK3C2A
35prostacyclin469.0PIK3C2A, ELANE, CTSG
36creatinine469.0CTSG, SERPINA3, PIK3C2A
37thyroxine46 259.9ELANE, SERPINA3, PIK3C2A
38lactate468.9PIK3C2A, ELANE, CTSG
39acetylcholine46 52 30 25 1212.9ELANE, SERPINA3, PIK3C2A
40arginine468.9CTSG, SERPINA3, PIK3C2A
41cyclosporin a46 30 6210.8CSF3, ELANE, PIK3C2A
42gold468.8SERPINA3, PIK3C2A
43fmlp468.8SERPINA3, ELANE, CSF3, CTSG
44retinoic acid46 259.7CTSG, CSF3, ELANE, SERPINA3
45alanine468.7CTSG, SERPINA3, PIK3C2A
46oxygen46 259.5ELANE, SERPINA3, PIK3C2A
47epinephrine46 25 1210.5PIK3C2A, ELANE, CSF3, CTSG
48fibrinogen468.5CTSG, ELANE, SERPINA3, PIK3C2A
49heparin46 30 25 1211.5PIK3C2A, SERPINA3, ELANE, CTSG
50superoxide46 259.0CTSG, CSF3, ELANE, SERPINA3, PIK3C2A

GO Terms for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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17Gene 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
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