MCID: ACT100
MIFTS: 48

Acute Febrile Neutrophilic Dermatosis malady

Rare diseases, Skin diseases categories

Aliases & Classifications for Acute Febrile Neutrophilic Dermatosis

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Sources:
41NIH Rare Diseases, 43Novoseek, 47Orphanet, 60UMLS, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
See all sources

Acute Febrile Neutrophilic Dermatosis, Aliases & Descriptions:

Name: Acute Febrile Neutrophilic Dermatosis 41 47
Sweet Syndrome 41 47 60
Neutrophilic Dermatosis, Acute Febrile 41
 
Gomm Button Disease 41
Sweets Syndrome 43
Ss 41


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 47 
Rare skin diseases


Characteristics (Orphanet epidemiological data):

47
sweet syndrome:
Prevalence: <1/1000000 (Worldwide); Age of onset: Adult


External Ids:

Orphanet47 3243
MESH via Orphanet34 D016463
ICD10 via Orphanet26 L98.2
UMLS via Orphanet61 C0085077

Summaries for Acute Febrile Neutrophilic Dermatosis

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NIH Rare Diseases:41 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 based summary: Acute Febrile Neutrophilic Dermatosis, also known as sweet syndrome, is related to leukemia and vasculitis, and has symptoms including skin rash, hypermelanotic macule and splenomegaly. 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 neutrophil, skin and bone, and related mouse phenotypes are immune system and hematopoietic system.

Related Diseases for Acute Febrile Neutrophilic Dermatosis

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

(show top 50)    (show all 156)
idRelated DiseaseScoreTop Affiliating Genes
1leukemia30.9CTSG, CSF3, SERPINA3
2vasculitis30.7CTSG, ELANE
3glomerulonephritis30.0CTSG, ELANE
4rheumatoid arthritis29.9SERPINA3, ELANE, CTSG
5pneumonia29.3CTSG, CSF3, ELANE, PIK3C2A
6lupus erythematosus10.6
7myelodysplastic syndrome10.6
8adenocarcinoma10.6
9congenital heart block10.6
10pyoderma gangrenosum10.5
11prostatitis10.5
12pyoderma10.5
13scleritis10.5
14systemic lupus erythematosus10.5
15cutaneous lupus erythematosus10.5
16subacute cutaneous lupus erythematosus10.4
17capillary leak syndrome10.4ELANE
18breast cancer10.3
19myocardial infarction10.3
20multiple myeloma10.3
21arthritis10.3
22relapsing polychondritis10.3
23dressler's syndrome10.3
24nodular episcleritis10.3
25cutis laxa10.3
26coccidioidomycosis10.3
27mixed connective tissue disease10.3
28anterior scleritis10.3
29cellulitis10.3
30connective tissue disease10.3
31fasciitis10.3
32influenza10.3
33myeloma10.3
34panniculitis10.3
35polyneuropathy10.3
36prostate adenocarcinoma10.3
37transitional cell carcinoma10.3
38essential thrombocythemia10.3
39rheumatoid vasculitis10.3
40premature ejaculation10.3
41sickle cell disease10.3
42sarcoma10.2
43sickle cell anemia10.2
44sjogren's syndrome10.2
45pulmonary emphysema10.2CTSG, ELANE
46acute respiratory distress syndrome10.1CTSG, ELANE
47extrinsic allergic alveolitis10.1CTSG, ELANE
48gingivitis10.1ELANE, CTSG
49neutropenia, cyclic10.1ELANE, CSF3
50cholangitis, primary sclerosing10.1CTSG, ELANE

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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Symptoms:

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

HPO human phenotypes related to Acute Febrile Neutrophilic Dermatosis:

(show all 23)
id Description Frequency HPO Source Accession
1 skin rash hallmark (90%) HP:0000988
2 hypermelanotic macule hallmark (90%) HP:0001034
3 splenomegaly hallmark (90%) HP:0001744
4 leukocytosis hallmark (90%) HP:0001974
5 migraine hallmark (90%) HP:0002076
6 arthralgia hallmark (90%) HP:0002829
7 myalgia hallmark (90%) HP:0003326
8 abnormality of temperature regulation hallmark (90%) HP:0004370
9 skin ulcer hallmark (90%) HP:0200042
10 hyperkeratosis typical (50%) HP:0000962
11 renal insufficiency occasional (7.5%) HP:0000083
12 proteinuria occasional (7.5%) HP:0000093
13 abnormality of the oral cavity occasional (7.5%) HP:0000163
14 hematuria occasional (7.5%) HP:0000790
15 thrombocytopenia occasional (7.5%) HP:0001873
16 anemia occasional (7.5%) HP:0001903
17 malabsorption occasional (7.5%) HP:0002024
18 pulmonary infiltrates occasional (7.5%) HP:0002113
19 recurrent respiratory infections occasional (7.5%) HP:0002205
20 abnormal blistering of the skin occasional (7.5%) HP:0008066
21 inflammatory abnormality of the eye occasional (7.5%) HP:0100533
22 glomerulopathy occasional (7.5%) HP:0100820
23 pustule occasional (7.5%) HP:0200039

Drugs & Therapeutics for Acute Febrile Neutrophilic Dermatosis

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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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MalaCards organs/tissues related to Acute Febrile Neutrophilic Dermatosis:

31
Neutrophil, Skin, Bone, Eye, Liver, Lung, Prostate, Bone marrow, Heart, Breast, Myeloid

Animal Models for Acute Febrile Neutrophilic Dermatosis or affiliated genes

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MGI Mouse Phenotypes related to Acute Febrile Neutrophilic Dermatosis:

35
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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Articles related to Acute Febrile Neutrophilic Dermatosis:

(show top 50)    (show all 114)
idTitleAuthorsYear
1
Sight-threatening ocular manifestations of acute febrile neutrophilic dermatosis (Sweet's syndrome). (24603349)
2014
2
Azathioprine-induced acute febrile neutrophilic dermatosis (Sweet's syndrome). (23955987)
2013
3
Acute febrile neutrophilic dermatosis (Sweet's syndrome). (23207661)
2013
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
Acute febrile neutrophilic dermatosis (Sweet's syndrome): a study of 15 cases in Iran. (17550553)
2007
7
Acute febrile neutrophilic dermatosis: a histopathologic study of 31 cases with review of literature. (17414432)
2007
8
From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research. (17097401)
2006
9
Subcutaneous Sweet's syndrome: a variant of acute febrile neutrophilic dermatosis that is included in the histopathologic differential diagnosis of neutrophilic panniculitis. (15858502)
2005
10
What is your diagnosis? Sweet Syndrome (acute febrile neutrophilic dermatosis). (14655771)
2003
11
Heart failure in acute febrile neutrophilic dermatosis. (14585639)
2003
12
Acute febrile neutrophilic dermatosis (Sweet's syndrome) with nodular episcleritis and polyneuropathy. (11982648)
2002
13
Acute febrile neutrophilic dermatosis following tuberculous infection. (12568224)
2002
14
Sweet's syndrome (acute febrile neutrophilic dermatosis). (10673451)
1999
15
Scleritis associated with acute febrile neutrophilic dermatosis (Sweet's syndrome) (9602638)
1998
16
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with relapsing polychondritis. (9892977)
1998
17
Acute febrile neutrophilic dermatosis (Sweet's syndrome) of the head and neck. (9664250)
1998
18
Concurrent Sweet's syndrome (acute febrile neutrophilic dermatosis), erythema nodosum and sarcoidosis. (9330058)
1997
19
Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome. (8621829)
1996
20
Multiple erythematous tender papules and nodules in an 11-month-old boy. Sweet syndrome (SS) (acute febrile neutrophilic dermatosis). (8961884)
1996
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
Acute febrile neutrophilic dermatosis (Sweet's syndrome). A report of 2 cases. (2928884)
1989
29
Acute febrile neutrophilic dermatosis (Sweet's syndrome) and adenocarcinoma of the rectum. (3214949)
1988
30
Pyoderma gangrenosum versus acute febrile neutrophilic dermatosis (Sweet's syndrome) (3674086)
1987
31
Rapid onset of pustular plaques. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (3827286)
1987
32
Pulmonary involvement in Sweet's syndrome (acute febrile neutrophilic dermatosis). Preleukemic and leukemic phases of acute myelogenous leukemia. (3465510)
1986
33
Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with post-myocardial infarction syndrome (Dressler's syndrome). (2411241)
1985
34
Acute febrile neutrophilic dermatosis (Sweet's syndrome) and the related conditions of &quot;bowel bypass&quot; syndrome and bullous pyoderma gangrenosum. (3912087)
1985
35
Acute febrile neutrophilic dermatosis and abnormal bone marrow chromosomes as a marker for preleukemia. (2408427)
1985
36
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Response to dapsone. (6696481)
1984
37
Colchicine in the treatment of acute febrile neutrophilic dermatosis (Sweet's syndrome). (6821647)
1983
38
Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders. (6572088)
1983
39
Fatal cardiovascular disease and cutis laxa following acute febrile neutrophilic dermatosis. (6822929)
1983
40
Sweet's syndrome (acute febrile neutrophilic dermatosis): report of two cases. (6841165)
1983
41
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with hairy-cell leukemia. (6625120)
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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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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Pathways related to Acute Febrile Neutrophilic Dermatosis according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.4CTSG, ELANE

Compounds for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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Compounds related to Acute Febrile Neutrophilic Dermatosis according to GeneCards Suite gene sharing:

(show top 50)    (show all 58)
idCompoundScoreTop Affiliating Genes
1elastatinal439.8ELANE, CTSG
24-nitroanilide439.8ELANE, CTSG
3saccharin43 210.8CTSG, ELANE
4(4-aminophenyl)mercuric acetate439.8ELANE, CTSG
5thermolysin439.8CTSG, ELANE
6pmsf439.8ELANE, CTSG
7hocl439.8ELANE, CTSG
8contrapsin439.8ELANE, SERPINA3
9epsilon aminocaproic acid439.8CTSG, ELANE
10pegfilgrastim43 1210.8CSF3, ELANE
11phosphoramidon43 1210.7ELANE, CTSG
12propylthiouracil43 2 1211.7ELANE, CSF3
13filgrastim43 1210.7CSF3, ELANE
14cytochalasin b439.7ELANE, CTSG
15gm-csf43 2810.7CSF3, ELANE
16kininogen439.7CTSG, ELANE
17aprotinin43 1210.6CTSG, ELANE
18phosphocreatine43 2410.5PIK3C2A, CTSG
19leukotriene b443 59 2411.5ELANE, CTSG
20sodium dodecylsulfate439.5CTSG, ELANE
21xanthine43 2410.5CTSG, PIK3C2A
22malondialdehyde439.4PIK3C2A, CTSG
23thromboxane a243 2410.4CTSG, PIK3C2A
24melphalan43 49 1211.4CSF3, PIK3C2A
25nitroglycerin43 1210.3ELANE, PIK3C2A
26chymostatin439.3SERPINA3, ELANE, CTSG
27diisopropylfluorophosphate439.3CTSG, ELANE, SERPINA3
28n-formylmethionyl-leucylphenylalanine439.3CTSG, CSF3, ELANE
29endotoxin439.3CTSG, CSF3, ELANE
30valine439.2CSF3, ELANE, SERPINA3
31urea43 24 1211.1PIK3C2A, SERPINA3, CTSG
32sodium nitroprusside439.0PIK3C2A, ELANE
33prednisolone43 28 1211.0CSF3, PIK3C2A
34methotrexate49 43 1211.0CSF3, SERPINA3, PIK3C2A
35prostacyclin439.0PIK3C2A, ELANE, CTSG
36creatinine439.0CTSG, SERPINA3, PIK3C2A
37thyroxine43 249.9ELANE, SERPINA3, PIK3C2A
38lactate438.9PIK3C2A, ELANE, CTSG
39acetylcholine43 49 28 24 1212.9ELANE, SERPINA3, PIK3C2A
40arginine438.9CTSG, SERPINA3, PIK3C2A
41cyclosporin a43 28 5910.8CSF3, ELANE, PIK3C2A
42gold438.8SERPINA3, PIK3C2A
43fmlp438.8SERPINA3, ELANE, CSF3, CTSG
44retinoic acid43 249.7CTSG, CSF3, ELANE, SERPINA3
45alanine438.7CTSG, SERPINA3, PIK3C2A
46oxygen43 249.5ELANE, SERPINA3, PIK3C2A
47epinephrine43 24 1210.5PIK3C2A, ELANE, CSF3, CTSG
48fibrinogen438.5CTSG, ELANE, SERPINA3, PIK3C2A
49heparin43 28 24 1211.5PIK3C2A, SERPINA3, ELANE, CTSG
50superoxide43 249.0CTSG, CSF3, ELANE, SERPINA3, PIK3C2A

GO Terms for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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Cellular components related to Acute Febrile Neutrophilic Dermatosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:00301419.4CTSG, ELANE
2extracellular vesicular exosomeGO:00700627.8CTSG, ELANE, SERPINA3, PIK3C2A

Biological processes related to Acute Febrile Neutrophilic Dermatosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of immune responseGO:00507789.5CTSG, ELANE
2negative regulation of growth of symbiont in hostGO:00441309.4CTSG, ELANE
3proteolysisGO:00065089.4CTSG, ELANE
4extracellular matrix disassemblyGO:00226179.3ELANE, CTSG
5response to lipopolysaccharideGO:00324969.1CTSG, ELANE

Molecular functions related to Acute Febrile Neutrophilic Dermatosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:00042529.4CTSG, ELANE
2peptidase activityGO:00082339.3CTSG, ELANE
3heparin bindingGO:00082019.1CTSG, ELANE

Products for genes affiliated with Acute Febrile Neutrophilic Dermatosis

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Sources for Acute Febrile Neutrophilic Dermatosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet