SS
MCID: ACT100
MIFTS: 57

Acute Febrile Neutrophilic Dermatosis (SS) malady

Rare diseases, Skin diseases categories

Summaries for Acute Febrile Neutrophilic Dermatosis

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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 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.

Description from OMIM:46 608068

Aliases & Classifications for Acute Febrile Neutrophilic Dermatosis

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Sources:
42NIH Rare Diseases, 48Orphanet, 61UMLS, 46OMIM, 44Novoseek, 35MESH via Orphanet, 27ICD10 via Orphanet, 62UMLS via Orphanet
See all sources

Acute Febrile Neutrophilic Dermatosis, Aliases & Descriptions:

Name: Acute Febrile Neutrophilic Dermatosis 42 48
Sweet Syndrome 42 48 46 61
Neutrophilic Dermatosis, Acute Febrile 42 61
 
Gomm Button Disease 42 61
Sweets Syndrome 44 61
Ss 42


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 48 
Rare skin diseases


Characteristics (Orphanet epidemiological data):

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


External Ids:

OMIM46 608068
MESH via Orphanet35 D016463
ICD10 via Orphanet27 L98.2
UMLS via Orphanet62 C0085077

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/GeneDecks gene sharing:

(show top 50)    (show all 165)
idRelated DiseaseScoreTop Affiliating Genes
1leukemia30.9CSF3, CTSG, SERPINA3
2vasculitis30.7CTSG, ELANE
3sepsis30.4CSF3, ELANE, CTSG
4glomerulonephritis30.0ELANE, CTSG
5rheumatoid arthritis29.9CTSG, ELANE, SERPINA3
6pneumonia29.4ELANE, CSF3, CTSG, PIK3C2A
7lupus erythematosus10.6
8congenital heart block10.6
9myelodysplastic syndromes10.6
10adenocarcinoma10.6
11pyoderma gangrenosum10.5
12hairy cell leukemia10.5
13prostatitis10.5
14pyoderma10.5
15sarcoidosis10.5
16scleritis10.5
17systemic lupus erythematosus10.5
18cutaneous lupus erythematosus10.5
19subacute cutaneous lupus erythematosus10.4
20capillary leak syndrome10.4ELANE
21arthritis10.3
22multiple myeloma10.3
23myocardial infarction10.3
24relapsing polychondritis10.3
25nodular episcleritis10.3
26coccidioidomycosis10.3
27dressler's syndrome10.3
28cutis laxa10.3
29mixed connective tissue disease10.3
30anterior scleritis10.3
31breast cancer10.3
32cellulitis10.3
33connective tissue disease10.3
34fasciitis10.3
35influenza10.3
36myeloma10.3
37panniculitis10.3
38polyneuropathy10.3
39prostate adenocarcinoma10.3
40transitional cell carcinoma10.3
41essential thrombocythemia10.3
42rheumatoid vasculitis10.3
43premature ejaculation10.3
44sickle cell disease10.3
45amyloid tumor10.2SERPINA3, CTSG
46pulmonary emphysema10.2CTSG, ELANE
47adult respiratory distress syndrome10.2ELANE, CTSG
48sickle cell anemia10.2
49sjogren's syndrome10.2
50sarcoma10.2

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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Clinical features from OMIM:

608068

Symptoms:

 48 (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:

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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:

32
Neutrophil, Skin, Bone, Liver, Lung, Prostate, Bone marrow, 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:

36
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 115)
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
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
Concurrent Sweet's syndrome (acute febrile neutrophilic dermatosis), erythema nodosum and sarcoidosis. (9330058)
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
Acute febrile neutrophilic dermatosis (Sweet's syndrome). (8486457)
1993
21
Acute febrile neutrophilic dermatosis in association with erythema nodosum and sarcoidosis. (8440052)
1993
22
Acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with BehAset's disease. (1459764)
1992
23
Acute febrile neutrophilic dermatosis (Sweet's syndrome) in metastatic breast cancer. (1515281)
1992
24
Acute bullous hemorrhagic eruption. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (2181942)
1990
25
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
26
Acute febrile neutrophilic dermatosis (Sweet's syndrome). Association with rheumatoid vasculitis. (2757408)
1989
27
Fever and painful plaques on the face, back, and extremities. Acute febrile neutrophilic dermatosis (Sweet's syndrome). (2673050)
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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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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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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Compounds related to Acute Febrile Neutrophilic Dermatosis according to GeneCards/GeneDecks:

(show top 50)    (show all 58)
idCompoundScoreTop Affiliating Genes
1elastatinal449.8ELANE, CTSG
24-nitroanilide449.8ELANE, CTSG
3saccharin44 310.8CTSG, ELANE
4(4-aminophenyl)mercuric acetate449.8ELANE, CTSG
5thermolysin449.8CTSG, ELANE
6pmsf449.8ELANE, CTSG
7hocl449.8ELANE, CTSG
8contrapsin449.8ELANE, SERPINA3
9epsilon aminocaproic acid449.8CTSG, ELANE
10pegfilgrastim44 1210.8CSF3, ELANE
11phosphoramidon44 1210.7ELANE, CTSG
12propylthiouracil44 3 1211.7ELANE, CSF3
13filgrastim44 1210.7CSF3, ELANE
14cytochalasin b449.7ELANE, CTSG
15gm-csf44 2910.7CSF3, ELANE
16kininogen449.7CTSG, ELANE
17aprotinin44 1210.6CTSG, ELANE
18phosphocreatine44 2510.5PIK3C2A, CTSG
19leukotriene b444 60 2511.5ELANE, CTSG
20sodium dodecylsulfate449.5CTSG, ELANE
21xanthine44 2510.5CTSG, PIK3C2A
22malondialdehyde449.4PIK3C2A, CTSG
23thromboxane a244 2510.4CTSG, PIK3C2A
24melphalan44 50 1211.4CSF3, PIK3C2A
25nitroglycerin44 1210.3ELANE, PIK3C2A
26chymostatin449.3SERPINA3, ELANE, CTSG
27diisopropylfluorophosphate449.3CTSG, ELANE, SERPINA3
28n-formylmethionyl-leucylphenylalanine449.3CTSG, CSF3, ELANE
29endotoxin449.3CTSG, CSF3, ELANE
30valine449.2CSF3, ELANE, SERPINA3
31urea44 25 1211.1PIK3C2A, SERPINA3, CTSG
32sodium nitroprusside449.0PIK3C2A, ELANE
33prednisolone44 29 1211.0CSF3, PIK3C2A
34methotrexate50 44 1211.0CSF3, SERPINA3, PIK3C2A
35prostacyclin449.0PIK3C2A, ELANE, CTSG
36creatinine449.0CTSG, SERPINA3, PIK3C2A
37thyroxine44 259.9ELANE, SERPINA3, PIK3C2A
38lactate448.9PIK3C2A, ELANE, CTSG
39acetylcholine44 50 29 25 1212.9ELANE, SERPINA3, PIK3C2A
40arginine448.9CTSG, SERPINA3, PIK3C2A
41cyclosporin a44 29 6010.8CSF3, ELANE, PIK3C2A
42gold448.8SERPINA3, PIK3C2A
43fmlp448.8SERPINA3, ELANE, CSF3, CTSG
44retinoic acid44 259.7CTSG, CSF3, ELANE, SERPINA3
45alanine448.7CTSG, SERPINA3, PIK3C2A
46oxygen44 259.5ELANE, SERPINA3, PIK3C2A
47epinephrine44 25 1210.5PIK3C2A, ELANE, CSF3, CTSG
48fibrinogen448.5CTSG, ELANE, SERPINA3, PIK3C2A
49heparin44 29 25 1211.5PIK3C2A, SERPINA3, ELANE, CTSG
50superoxide44 259.0CTSG, 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/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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Sources for Acute Febrile Neutrophilic Dermatosis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
29IUPHAR
30KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet