MCID: WLL006
MIFTS: 55

Wells Syndrome malady

Skin category

Summaries for Wells Syndrome

Sources:
43NIH Rare Diseases, 3CDC, 64Wikipedia, 33MalaCards
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CDC:3 Many people in the United States receive their water from private ground water wells. EPA regulations that protect public drinking water systems do not apply to privately owned wells. As a result, owners of private wells are responsible for ensuring that their water is safe from contaminants. Here you may find information on the basics of wells, proper methods of siting and location for wells, all about testing and how often to test a well, proper treatment of wells and maintenance of wells , information on well retirement, common diseases and contaminants associated with wells, emergency treatment of wells, and answers to frequently asked questions about wells.

MalaCards: Wells Syndrome, also known as eosinophilic cellulitis, is related to muckle-wells syndrome and ankyloblepharon-ectodermal defects-cleft lip/palate syndrome, and has symptoms including subcutaneous nodules/lipomas/tumefaction/swelling, cutaneous rash and erythema/erythematous lesions/erythroderma/polymorphous erythema. An important gene associated with Wells Syndrome is NLRP3 (NLR family, pyrin domain containing 3), and among its related pathways are Senescence and Autophagy and Spinal Cord Injury. The compounds p005 and tenidap have been mentioned in the context of this disorder. Affiliated tissues include skin, brain and kidney, and related mouse phenotypes are renal/urinary system and integument.

NIH Rare Diseases:43 Wells syndrome is a rare eosinophilic disorder characterized by itchy, burning, red, and inflamed areas on the skin often on the lower arm or leg. the lesions may be single or multiple. they are usually red at first (looking like cellulitis) then change to brown red, to blue gray, then greenish gray. the lesions are usually painful and are sometimes associated with a fever. they typically heal without scarring within weeks to months; however they often recur. the cause of wells syndrome is unknown; but some researchers think that wells syndrome is an autoimmune reaction. last updated: 12/14/2011

Wikipedia:64 Eosinophilic cellulitus (also known as \"Wells\' syndrome\" from George Crichton Wells who first... more...

Aliases & Classifications for Wells Syndrome

Sources:
43NIH Rare Diseases, 49Orphanet, 61UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
wells syndrome:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

wells syndrome 43 49 61
eosinophilic cellulitis 43 49
bullous cellulitis with eosinophilia 43
wells' syndrome 43


External Ids:

MESH via Orphanet36 C536693
ICD10 via Orphanet26 L98.3
UMLS via Orphanet62 C0343101
ICD1025 L98.3

Related Diseases for Wells Syndrome

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Wells Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 206)
idRelated DiseaseScoreTop Affiliating Genes
1muckle-wells syndrome31.1IL1B, IL1RAPL2, IL1R1, MEFV, TNFRSF1A, CASP1
2ankyloblepharon-ectodermal defects-cleft lip/palate syndrome30.4FGFR2, TP63
3ectodermal dysplasia30.2TNFRSF1A, TP63
4urticaria30.2NLRP3, CASP1, IL1RAPL2
5amyloidosis30.2TNFRSF1A, CRP, MEFV, IL1RAPL2
6adenocarcinoma30.0CASP1, GNB2L1, FGFR2, IL1RAPL2, TP63
7familial cold autoinflammatory syndrome29.9NLRP3, IL1B, IL1R1, MEFV, TNFRSF1A, CASP1
8uveitis29.7IL1R1, TNFRSF1A
9amyloidosis, secondary29.7CRP, MEFV
10asthma29.7IL1B, IL8, IL3
11arthropathy29.7NLRP3, CRP, IL1RAPL2, IL1B
12systemic lupus erythematosus29.7TNFRSF1A, CRP, IL1R1, IL1RAPL2
13ulcerative colitis29.7CASP1, CRP, IL1R1, IL1RAPL2, IL8, IL1B
14cellulitis10.6
15n syndrome10.5
16churg-strauss syndrome10.3
17folliculitis10.2
18eosinophilic pustular folliculitis10.2
19granulomatous dermatitis10.1
20hypereosinophilic syndrome10.1
21thromboangiitis obliterans10.1
22eosinophilic fasciitis10.1
23cutaneous mastocytoma10.1
24pericarditis10.0MEFV
25schnitzler syndrome10.0IL1R1
26ischemia10.0CASP1
27alopecia areata10.0IL1R1
28dermatitis10.0NLRP3
29colon cancer10.0CASP1
30palindromic rheumatism10.0MEFV
31cervical cancer10.0IL1R1
32influenza10.0CASP1
33polyarteritis nodosa10.0MEFV
34gingivitis10.0IL1B
35diabetic retinopathy10.0CASP1
36acquired immunodeficiency syndrome10.0IL1B
37down syndrome10.0CASP1
38hepatitis b10.0TNFRSF1A
39common variable immune deficiency10.0TNFRSF1A
40hepatitis10.0IL1RAPL2
41encephalitis10.0IL1B
42bronchiolitis10.0IL8
43dementia10.0IL1RAPL2
44crohn's disease10.0CRP
45allergic rhinitis10.0IL8
46insulinoma10.0CASP1
47rhinitis10.0IL8
48periodontal disease10.0CRP
49amyotrophic lateral sclerosis10.0CASP1
50pancreas disease10.0CASP1

Graphical network of the top 20 diseases related to Wells Syndrome:



Diseases related to wells syndrome

Clinical Features for Wells Syndrome

Sources:
49Orphanet
See all sources


Symptoms:

49 (show all 10)
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • cutaneous rash
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • follicular/erythematous/edematous papules/milium
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • pruritus/itching
  • cutaneous edema
  • urticaria
  • eosinophils anomalies/hypereosinophilia
  • cellulitis/panniculitis/pseudocellulitis/inflammation of subcutaneous tissue

Drugs & Therapeutics for Wells Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Wells Syndrome

Drug clinical trials:

Search ClinicalTrials for Wells Syndrome

Search NIH Clinical Center for Wells Syndrome

Search CenterWatch for Wells Syndrome

Genetic Tests for Wells Syndrome

Anatomical Context for Wells Syndrome

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Wells Syndrome:

33
Skin, Brain, Kidney, Colon, Thyroid, Salivary gland, Myeloid, Monocytes, T cells, B cells

Animal Models for Wells Syndrome or affiliated genes

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

Publications for Wells Syndrome

Sources:
51PubMed
See all sources

Articles related to Wells Syndrome:

(show top 50)    (show all 185)
idTitleAuthorsYear
1
Somatic NLRP3 mosaicism in Muckle-Wells syndrome. A genetic mechanism shared by different phenotypes of cryopyrin-associated periodic syndromes. (24326009)
2013
2
Muckle-Wells syndrome: individualized, reflare-guided dosing of the orphan drug canakinumab. (23745225)
2013
3
Idiopathic bullous eosinophilic cellulitis (Wells syndrome) responsive to topical tacrolimus and antihistamine combination. (22565446)
2012
4
Diagnosis and management of eosinophilic cellulitis (Wells' syndrome): A case series and literature review. (23730155)
2012
5
Efficacy and safety of anakinra therapy in pediatric and adult patients with the autoinflammatory Muckle-Wells syndrome. (21360513)
2011
6
Eosinophilic annular erythema: an expression of the clinical and pathological polymorphism of Wells syndrome. (21920240)
2011
7
Muckle-Wells syndrome effectively treated with canakinumab: is the recommended dosing schedule mandatory? (21967869)
2011
8
Audiometric characteristics of a Dutch family with Muckle-Wells syndrome. (21810457)
2011
9
Wells syndrome (eosinophilic cellulitis): a clinical imitator of bacterial cellulitis. (21779422)
2011
10
A patient with Wells' syndrome. (20500167)
2010
11
High responses of peripheral lymphocytes to mosquito salivary gland extracts in patients with Wells syndrome. (20542179)
2010
12
A case report of Wells' syndrome in a celiac patient. (20872333)
2010
13
Muckle-Wells syndrome/neonatal-onset multisystem inflammatory disease overlap associated with myelodysplasia and cerebrovascular accident. (20456407)
2010
14
Craniofacial and anthropometric phenotype in ankyloblepharon-ectodermal defects-cleft lip/palate syndrome (Hay-Wells syndrome) in a cohort of 17 patients. (19676059)
2009
15
Impetiginized Wells' syndrome in a patient with chronic lymphocytic leukaemia. (19438566)
2009
16
Eosinophilic annular erythema: a subset of Wells' syndrome or a distinct entity? (18638225)
2008
17
Anakinra improves sensory deafness in a Japanese patient with Muckle-Wells syndrome, possibly by inhibiting the cryopyrin inflammasome. (18311804)
2008
18
Lasting remission of a Muckle-Wells syndrome with CIAS-1 mutation using half-dose anakinra. (17892965)
2007
19
Wells' syndrome after primoinfection by parvovirus B19 in a child. (17224386)
2007
20
Churg-Strauss syndrome and Wells syndrome: coincidence or pathogenetic association? A new case report. (17428365)
2007
21
Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra. (16646042)
2006
22
Rapp-Hodgkin ectodermal dysplasia syndrome: the clinical and molecular overlap with Hay-Wells syndrome. (16532463)
2006
23
Relapse of Wells' syndrome in a child after tetanus-diphtheria immunization. (16631949)
2006
24
Hay-Wells syndrome (AEC): a case report. (16910923)
2006
25
Reflections on eosinophils and flame figures: where there's smoke there's not necessarily Wells syndrome. (16983010)
2006
26
Bullous &quot;cellulitis&quot; with eosinophilia: case report and review of Wells' syndrome in childhood. (15995016)
2005
27
Eosinophilic cellulitis of papulonodular presentation (Wells' syndrome). (15752298)
2005
28
A neonate with denuded skin: Hay-Wells syndrome. (15262880)
2004
29
CIAS1 mutation in a patient with overlap between Muckle-Wells and chronic infantile neurological cutaneous and articular syndromes. (12673085)
2003
30
Muckle-Wells syndrome? (11312446)
2001
31
Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle-Wells syndrome. (11687797)
2001
32
Eosinophil activation in Wells' syndrome demonstrated immunohistochemically with antibodies against eosinophilic cationic protein. (10721845)
2000
33
Wells syndrome associated with Churg-Strauss syndrome. (10954676)
2000
34
Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5. (10215782)
1999
35
Guess what? Wells' syndrome. (10094595)
1999
36
Wells' syndrome responsive to PUVA therapy. (9349363)
1997
37
Toxocariasis and Wells' syndrome: a causal relationship? (9529550)
1997
38
What syndrome is this? Ankyloblepharon-ectodermal defects--cleft lip and palate (Hay-Wells) syndrome. (9336818)
1997
39
Wells' syndrome: a pathogenic role for circulating CD4+CD7- T cells expressing interleukin-5 mRNA. (9217826)
1997
40
Eosinophilic cellulitis (Wells' syndrome) associated with ascariasis. (7976090)
1994
41
Autosomal dominant Muckle-Wells syndrome associated with cystinuria, ichthyosis, and aphthosis in a four-generation family. (7802040)
1994
42
Pathological case of the month. Wells' syndrome. (8249958)
1993
43
Wells' syndrome with lock jaw: report of a case in Thai patient. (1765748)
1991
44
Low-dose alternate-day prednisone for persistent Wells' syndrome. (2589856)
1989
45
Wells' syndrome. (2915071)
1989
46
Eosinophilic cellulitis (Wells' syndrome): ultrastructural study of a case with circulating immune complexes. (2969834)
1988
47
The histopathogenesis of the flame figure in Wells' syndrome based on five cases. (2426897)
1986
48
Eosinophilic cellulitis (Wells' syndrome) (4015127)
1985
49
Eosinophilic cellulitis (Wells' syndrome): histologic and clinical features in arthropod bite reactions. (6512050)
1984
50
Wells' syndrome: is there collagen damage in the flame figures? (6520258)
1984

Genetic Variations for Wells Syndrome

Expression for genes affiliated with Wells Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Wells Syndrome

Search GEO for disease gene expression data for Wells Syndrome.

Pathways for genes affiliated with Wells Syndrome

Sources:
38NCBI BioSystems Database, 54Reactome, 52QIAGEN, 30KEGG, 12EMD Millipore, 53R&D Systems
See all sources

Pathways related to Wells Syndrome according to GeneCards/GeneDecks:

(show all 31)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.1IL8
210.0IL1B, IL1R1
39.9IL1B, CASP1
4
Hide members
9.8MEFV, CASP1, NLRP3
5
Hide members
9.8FGFR2, TNFRSF1A
6
Hide members
9.7IL1R1, TNFRSF1A, CASP1
7
Hide members
9.7IL1B, IL1R1, CASP1
89.6IL1B, IL1R1, IL3
99.6IL1R1, IL8, IL1B
10
Immune response Bacterial infections in normal airways
Hide members
9.6IL1R1, IL8, IL1B
11
Hide members
9.6IL1B, IL8, IL1R1
129.6CASP1, IL8, IL1B
139.6CASP1, IL8, IL1B
149.5IL1B, IL8, IL3
15
Hide members
9.5FGFR2, GNB2L1
16
Hide members
9.4CASP1, TNFRSF1A, IL1R1, IL1B
17
Hide members
9.4CASP1, IL3, IL1R1, IL1B
18
Hide members
9.4IL1B, IL1R1, IL3, CASP1
199.4IL1B, IL8, CASP1, NLRP3
209.3TNFRSF1A, IL1R1, IL8, IL1B
21
Hide members
9.2IL1R1, IL3, FGFR2, TNFRSF1A
22
Hide members
9.2CASP1, TNFRSF1A, FGFR2, IL3
23
Hide members
9.1IL1B, IL8, MEFV, CASP1, NLRP3
24
Hide members
9.1NLRP3, CASP1, IL1R1, IL8, IL1B
25
Hide members
9.1CASP1, TNFRSF1A, IL3, IL1R1, IL1B
26
Hide members
9.0IL1B, IL1R1, FGFR2, TNFRSF1A, CASP1
27
Hide members
8.9TNFRSF1A, FGFR2, IL3, IL1R1, IL1B
28
Hide members
8.9CASP1, TNFRSF1A, FGFR2, IL3, IL1B
29
Hide members
8.6NLRP3, CASP1, GNB2L1, TNFRSF1A, IL8, IL1B
30
Hide members
8.6TNFRSF1A, FGFR2, IL3, IL1R1, IL8, IL1B
31
Hide members
8.5NLRP3, CASP1, MEFV, FGFR2, IL3, IL1R1

Compounds for genes affiliated with Wells Syndrome

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

Compounds related to Wells Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 129)
idCompoundScoreTop Affiliating Genes
1p0054510.5TP63
2tenidap45 2911.4IL1R1
3cycloheximide4510.4IL1RAPL2
4endotoxin4510.2MEFV, TNFRSF1A, CASP1
5diphenyleneiodonium4510.0CASP1, IL1B, IL1R1
6fmlp459.9IL1RAPL2, IL3
7tlck459.9CASP1, IL1B, IL8
8pentoxifylline45 1110.9IL1R1, CASP1, IL1RAPL2
9muramyl dipeptide45 2910.8CASP1, IL1B, IL8
10as 10160 4510.8IL1B, IL3, CASP1, IL1R1
11lxa4459.7IL3, IL8, IL1B
12mevalonate459.7NLRP3, MEFV, TNFRSF1A, CASP1
13peptidoglycan45 2910.6IL8, IL1R1, IL1B, CASP1
14poly(i-c)459.6IL8, IL1B, IL3, CASP1
15monosodium urate459.5IL1B, IL1R1, IL1RAPL2, IL8
16leflunomide50 45 1111.5IL1RAPL2, TNFRSF1A, IL1B, IL1R1
17polysulfone459.4CRP, IL1B, IL1R1, IL1RAPL2
18cuprophan459.3IL1R1, TNFRSF1A, IL1B, IL1RAPL2, CASP1
19rantes459.2IL1R1, TNFRSF1A, IL3, IL1RAPL2, IL1B
20mip 1alpha459.2IL8, IL1B, IL3
21infliximab45 50 1111.2TNFRSF1A, IL1B, IL1RAPL2, IL1R1, CRP
22il 10459.2IL1B, IL8, IL1RAPL2, IL1R1, IL3
23leukotriene b445 60 2411.1IL1B, IL1RAPL2, IL1R1, IL3, IL8
24sulfasalazine45 29 50 1112.1IL1B, IL1R1, CRP, IL1RAPL2, IL8
25dexamethasone45 50 29 1112.1CASP1, IL3, IL1R1, IL1RAPL2, AVP
26glutamate459.1TNFRSF1A, GNB2L1, IL1RAPL2, AVP
27sb 20358045 6010.1CASP1, IL8, IL1R1, CRP, IL1B, TNFRSF1A
28anakinra45 1110.0CASP1, CRP, IL1R1, IL1RAPL2, IL1B, NLRP3
29creatinine459.0IL1RAPL2, CASP1, TNFRSF1A, CRP, AVP
30methotrexate45 50 1111.0NLRP3, TNFRSF1A, CRP, IL1R1, IL1RAPL2, IL1B
31ivig458.9IL3, CRP, TNFRSF1A, IL1R1, IL1B, IL1RAPL2
32etanercept45 50 1110.9IL8, IL1RAPL2, IL1R1, TNFRSF1A, IL1B, CRP
33cyclophosphamide45 50 1110.9CRP, IL3, IL1RAPL2, IL1R1, FGFR2
34ribonucleic acid458.8CASP1, TNFRSF1A, FGFR2, IL1R1, IL1B, IL1RAPL2
35aspirin45 50 29 2411.8IL1R1, IL1B, CASP1, CRP, IL1RAPL2
36h2o2458.7TNFRSF1A, IL1RAPL2, IL1R1, IL3, FGFR2, CASP1
37actinomycin d458.7CASP1, TNFRSF1A, IL3, IL1R1, IL8, IL1B
38cyclosporin a45 29 6010.6IL8, IL1RAPL2, CASP1, TNFRSF1A, IL3, IL1R1
39neopterin458.6TNFRSF1A, CRP, IL1RAPL2, IL8, IL1R1, IL1B
40cisplatin45 50 60 1111.5FGFR2, IL1R1, TP63, IL3, TNFRSF1A, CASP1
41lactate458.5IL1RAPL2, CRP, CASP1, IL8, IL3, IL1R1
42n acetylcysteine458.4IL8, IL1R1, IL3, TNFRSF1A, CASP1, IL1B
43thymidine45 249.4IL1B, TP63, FGFR2, IL3, IL1R1, IL1RAPL2
44retinoic acid45 249.2CASP1, TP63, IL1B, IL1RAPL2, IL1R1, IL3
45testosterone45 60 11 2411.1CASP1, TP63, IL1RAPL2, IL1R1, IL3, FGFR2
46nitric oxide45 11 2410.1FGFR2, CASP1, IL1RAPL2, TNFRSF1A, IL1R1, IL8
47serine458.1IL1R1, TNFRSF1A, IL1RAPL2, IL8, GNB2L1, CASP1
48vegf457.9TP63, CASP1, IL1B, IL8, IL1RAPL2, IL1R1
49threonine457.7AVP, IL1RAPL2, IL1R1, IL3, FGFR2, TNFRSF1A
50tyrosine457.3IL1R1, GNB2L1, TNFRSF1A, MEFV, FGFR2, CASP1

GO Terms for genes affiliated with Wells Syndrome

Sources:
16Gene Ontology
See all sources

Cellular components related to Wells Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1NLRP3 inflammasome complexGO:0725599.7CASP1, NLRP3
2extracellular spaceGO:0056158.5CRP, IL3, IL8, IL1B, AVP, TNFRSF1A
3extracellular regionGO:0055767.4AVP, CASP1, TNFRSF1A, CRP, FGFR2, IL3

Biological processes related to Wells Syndrome according to GeneCards/GeneDecks:

(show all 14)
idNameGO IDScoreTop Affiliating Genes
1interleukin-1 beta productionGO:03261110.2IL1B, CASP1
2nucleotide-binding domain, leucine rich repeat containing receptor signaling pathwayGO:03587210.0CASP1, NLRP3, MEFV
3squamous basal epithelial stem cell differentiation involved in prostate gland acinus developmentGO:06052910.0FGFR2, TP63
4negative regulation of inflammatory responseGO:05072810.0TNFRSF1A, MEFV, NLRP3
5cellular response to organic substanceGO:0713109.9IL1B, CASP1
6cellular response to mechanical stimulusGO:0712609.8IL1B, TNFRSF1A, CASP1
7response to ATPGO:0331989.8IL1B, CASP1
8positive regulation of interleukin-1 beta secretionGO:0507189.7NLRP3, CASP1
9activation of cysteine-type endopeptidase activity involved in apoptotic processGO:0069199.5CASP1, GNB2L1, NLRP3
10response to ethanolGO:0454719.5TNFRSF1A, CRP, AVP
11immune responseGO:0069559.3IL1R1, IL3, IL8, IL1B
12cell-cell signalingGO:0072679.2AVP, IL1B, FGFR2, IL3
13inflammatory responseGO:0069549.0IL8, NLRP3, TNFRSF1A, CRP, MEFV, IL1B
14apoptotic processGO:0069159.0TNFRSF1A, IL1B, NLRP3, TP63, CASP1, FGFR2

Molecular functions related to Wells Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1interleukin-1 receptor activityGO:0049089.7IL1RAPL2, IL1R1

Products for genes affiliated with Wells Syndrome

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Wells Syndrome

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