MCID: KMR001
MIFTS: 51

Kimura Disease malady

Rare diseases, Bone diseases, Skin diseases, Immune diseases categories
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Summaries for Kimura Disease

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43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 Kimura disease is a rare, benign, chronic disorder that causes inflammation of tissue (nodules) under the skin of the head or neck.  these nodules tend to recur despite treatment. the cause of this condition is unknown, but may be due to an immune response. last updated: 1/20/2011

MalaCards: Kimura Disease, also known as angiolymphoid hyperplasia with eosinophilia, is related to skin disease and mycosis fungoides. An important gene associated with Kimura Disease is RNASE3 (ribonuclease, RNase A family, 3), and among its related pathways are Calcineurin-regulated NFAT-dependent transcription in lymphocytes and OX40 Pathway. The compounds tolnaftate and loteprednol have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and kidney, and related mouse phenotypes are respiratory system and immune system.

Aliases & Classifications for Kimura Disease

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8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 58SNOMED-CT, 35MeSH, 40NCIt, 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: Bone diseases, Skin diseases, Immune diseases


Characteristics (Orphanet epidemiological data):

49
kimura disease:
Prevalence: <1/1000000; Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

kimura disease 8 43 10 45 49 62
angiolymphoid hyperplasia with eosinophilia 43
eosinophilic hyperplastic lymphogranuloma 43
eosinophilic lymphofollicular granuloma 43
disorder characterized by eosinophilia 62
eosinophilic granuloma of soft tissue 43
eosinophilic lymphofolliculosis 43
eosinophilic lymphogranuloma 49
kimura's disease 8


External Ids:

Disease Ontology8 DOID:7365
MeSH35 D000796
NCIt40 C26867
MESH via Orphanet36 D000796
ICD10 via Orphanet26 D21, I89.8, L92.8
SNOMED-CT via Orphanet59 399894006
UMLS via Orphanet63 C0033838

Related Diseases for Kimura Disease

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Sources:
17GeneCards, 18GeneDecks
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Diseases related to Kimura Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 97)
idRelated DiseaseScoreTop Affiliating Genes
1skin disease30.5RNASE3, IGHE, IL4, IL5
2mycosis fungoides30.1IFNA1, IL5
3eosinophilia30.0IGES, IGHE, RNASE3, PRG2, IL4, IL5
4hemangioma10.8
5histiocytoid hemangioma10.7
6peripheral t-cell lymphoma10.5
7aneurysm10.5
8burns10.4
9follicular mucinosis10.4
10arteriovenous malformation10.4
11varicocele10.4
12angiosarcoma10.4
13atherosclerosis10.4
14scabies10.4
15bowenoid papulosis10.4
16prurigo nodularis10.4
17angiomatosis10.4
18nephrotic syndrome10.3
19temporal arteritis10.2
20cervicitis10.2
21juvenile temporal arteritis10.2
22bronchitis10.1RNASE3
23myelofibrosis10.1IFNA1
24keratoconjunctivitis10.1RNASE3, IL5
25chronic eosinophilic pneumonia10.1IL5, RNASE3
26giant papillary conjunctivitis10.1IL5, RNASE3
27eosinophilic gastroenteritis10.1IGHE, IL5
28onchocerciasis10.1RNASE3, IL5
29churg-strauss syndrome10.1IL5, RNASE3
30paragonimiasis10.1IGHE, IL5
31pneumonia10.1IL5, IGES
32anisakiasis10.1IGHE, RNASE3
33toxocariasis10.1IGHE, RNASE3
34kidney cancer10.1IFNA1
35contact dermatitis10.1IGHE, IL5
36filariasis10.1IGHE, IL5
37strongyloidiasis10.1IGHE, IL5
38dermatographia10.1IGHE, PRG2
39atopic dermatitis10.0RNASE3, IL4
40esophagitis10.0IL5, PRG2
41cutaneous leishmaniasis10.0IL5, IL4
42conjunctivitis10.0RNASE3, IGHE, IGES
43immunodeficiency with hyper igm type 110.0IL4, IGHE
44leishmaniasis10.0IL5, IL4
45bronchiectasis10.0IGES, IGHE, RNASE3
46pulmonary tuberculosis10.0IL4, IGES
47otitis media10.0RNASE3, IGES, IGHE
48respiratory failure10.0IGHE, IGES, RNASE3
49helminthiasis10.0IL5, IGHE, RNASE3
50eosinophilic pneumonia10.0IGHE, IL5, RNASE3

Graphical network of the top 20 diseases related to Kimura Disease:



Diseases related to kimura disease

Symptoms for Kimura Disease

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Drugs & Therapeutics for Kimura Disease

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

Search ClinicalTrials for Kimura Disease

Search NIH Clinical Center for Kimura Disease

Genetic Tests for Kimura Disease

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Anatomical Context for Kimura Disease

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Sources:
33MalaCards
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MalaCards organs/tissues related to Kimura Disease:

33
Skin, Bone, Kidney, Lymph node, Colon, Salivary gland

Animal Models for Kimura Disease or affiliated genes

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Sources:
37MGI
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MGI Mouse Phenotypes related to Kimura Disease:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.1PRG2, IL4, IL5, KIT
2MP:00053877.8KIT, IL5, IL4, PRG2, IGHE
3MP:00053977.7KIT, IL5, IL4, PRG2, IGHE

Publications for Kimura Disease

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Sources:
52PubMed
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Articles related to Kimura Disease:

(show top 50)    (show all 67)
idTitleAuthorsYear
1
Kimura Disease of the Epiglottis: A Case Report and Review of Literature. (23907346)
2013
2
The false-positive finding of left pulmonary Kimura disease on 18F-FDG PET/CT. (23510880)
2013
3
Kimura disease involving a caruncle. (23543681)
2013
4
Kimura Disease: rare cause of a slowly progressive orbital mass. (24119195)
2013
5
Elevated numbers of cells producing interleukin-5 and interleukin-10 in a boy with Kimura disease. (22627370)
2012
6
Kimura disease: CT and MR imaging findings. (22173767)
2012
7
Epitrochlear mass in a patient on maintenance hemodialysis--Kimura disease. (22280213)
2012
8
Juvenile temporal arteritis with perifollicular lymphoid proliferation resembling Kimura disease. Report of a case. (21182505)
2011
9
Kimura disease in the parotid gland. (21239930)
2011
10
Kimura disease with necrosis of the limbs and mononeuritis multiplex. (21418278)
2011
11
Treatment of Kimura disease with intravenous immunoglobulin. (22106083)
2011
12
Head and neck MRI of Kimura disease. (21849365)
2011
13
Kimura disease: An unusual cause of head and neck masses. Report of 2 cases. (20155679)
2010
14
Thrombotic storm in Kimura disease. (19468829)
2010
15
Renal epitelial neoplasia associated with Kimura disease. (20876951)
2010
16
Kimura disease: a case report and review of the Chinese literature. (19052471)
2009
17
Thrombosis of temporal artery and renal vein in Kimura-disease-related nephrotic syndrome. (18066701)
2009
18
Kimura disease of the eyelid in an Indian man. (19594570)
2009
19
Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab. (19786854)
2009
20
Kimura disease of the epiglottis. (20614304)
2008
21
Kimura disease. (19098588)
2008
22
Kimura disease: review of the literature. (18808562)
2008
23
Simultaneous presentation of Kimura disease and angiolymphoid hyperplasia with eosinophilia. (18645439)
2008
24
Kimura disease of extremity: unusual manifestation in a long bone. (18457980)
2008
25
Clinical Challenges and Images in GI: image 3. Colonic involvement in Kimura disease. (18694752)
2008
26
Kimura disease: an unusual cause of cervical lymphadenopathy with salivary gland involvement. (18076832)
2007
27
Kimura disease: diagnostic imaging findings and surgical treatment. (17912083)
2007
28
Kimura disease with proliferative squamous metaplasia: an unusual finding and a potential diagnostic pitfall. (18306548)
2007
29
Kimura disease: diagnosis and prognostic factors. (17666261)
2007
30
Kimura disease in an African patient. (16213070)
2006
31
Synchronous unilateral renal cell carcinoma and Kimura disease of the kidney. (16979713)
2006
32
Kimura disease of the eyelid: a clinicopathologic study with electron microscopic observations. (17117118)
2006
33
Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease. (15888362)
2005
34
Focal segmental glomerulosclerosis associating Kimura disease. (15957554)
2005
35
Vincristine treatment of nephrotic syndrome complicated by Kimura disease. (15690191)
2005
36
Radiology quiz case 2: Kimura disease. (15492180)
2004
37
Bilateral lacrimal gland involvement with Kimura disease in an African American male. (15197070)
2004
38
Pranlukast regulates tumour growth by attenuating IL-4 production in Kimura disease. (15088141)
2004
39
Kimura disease: a clinicopathologic study of 21 cases. (15087670)
2004
40
Kimura disease in a patient with renal allograft failure secondary to chronic rejection. (12883971)
2003
41
Kimura disease. (14606173)
2003
42
Juvenile temporal arteritis is a manifestation of Kimura disease. (11803281)
2002
43
Steroid-sensitive anemia in a boy on dialysis--an association with Kimura disease. (11149107)
2000
44
Nephrotic syndrome associated with Kimura disease. (10872190)
2000
45
A case of Kimura disease treated with interferon and general corticoid therapy]. (10422145)
1999
46
Kimura disease of the orbit and ocular adnexa. (10466591)
1999
47
Pathologic quiz case 1. Kimura disease. (8703396)
1996
48
Kimura disease involving parotid gland and cervical nodes: CT and MR findings. (1545036)
1992
49
CT of Kimura disease. (2505557)
1989
50
Subcutaneous angiolymphoid hyperplasia (Kimura disease). Report of a case. (1191016)
1975

Variations for Kimura Disease

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Expression for genes affiliated with Kimura Disease

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Sources:
2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Kimura Disease

Search GEO for disease gene expression data for Kimura Disease.

Pathways for genes affiliated with Kimura Disease

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Sources:
50PathCards, 38NCBI BioSystems Database, 53QIAGEN, 12EMD Millipore, 54R&D Systems, 30KEGG, 60Thomson Reuters
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Pathways related to Kimura Disease according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Calcium signaling in the CD4+ TCR pathway38
9.7IL4, IL5
29.7IL4, IL5
39.7IL5, IL4
49.7IL5, IL4
59.7IL5, IL4
69.7IL5, IL4
79.7IL5, IL4
89.2IL4, IL5, KIT
99.2IFNA1, IL4, IL5
10
Show member pathways
Type III interferon signaling38
9.2IL5, IL4, IFNA1
119.1KIT, IL4, IFNA1
12
Show member pathways
Immune response BCR pathway60
Fc-epsilon receptor I signaling in mast cells38
9.1IL5, IL4, IGHE
139.1IL5, IL4, IGHE
14
Show member pathways
8.7IFNA1, IL4, IL5, KIT
15
Show member pathways
8.7IFNA1, IL4, IL5, KIT
16
Show member pathways
8.4PRG2, IL4, IL5, KIT
17
Show member pathways
8.0IL5, IL4, IFNA1, PRG2, RNASE3

Compounds for genes affiliated with Kimura Disease

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Sources:
45Novoseek, 11DrugBank, 29IUPHAR, 51PharmGKB, 24HMDB, 61Tocris Bioscience
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Compounds related to Kimura Disease according to GeneCards/GeneDecks:

(show top 50)    (show all 119)
idCompoundScoreTop Affiliating Genes
1tolnaftate459.9IL4, IL5
2loteprednol45 1110.8IL5, IL4
3pranlukast45 29 1111.7IL4, IL5, RNASE3
4salmeterol45 51 29 1112.7RNASE3, IL4, IL5
5mometasone45 1110.6IL4, IL5
6unicap459.6IL5, IGHE, RNASE3
7beclomethasone dipropionate459.6RNASE3, IL5, IGHE
8glatiramer acetate45 1110.6IFNA1, IL4, IL5
9toluene diisocyanate459.5RNASE3, IL5, IGHE
10sodium cromoglycate459.5IL5, RNASE3, IGHE
11cetirizine45 29 24 1112.5IL5, RNASE3, IGHE
12il-12459.5IL5, IL4, IFNA1
13ketotifen45 1110.5IGHE, IL5, RNASE3
14omalizumab45 1110.5IL5, IGHE, IL4
15ovomucoid459.5IL4, IGHE, IL5
16methylcellulose459.5IL5, IL4, KIT
17polyinosinic-polycytidylic acid459.5IFNA1, IL4, IL5
18salbutamol45 51 29 24 1113.5IL5, RNASE3, IGHE
192-chlorodeoxyadenosine459.5IFNA1, KIT, IL5
20poly(i-c)459.5IFNA1, IL5, IL4
21itraconazole45 1110.4IL4, IL5, IGHE
22tarc459.3IGHE, IL4, IL5
23ltc4459.3PRG2, IL5, RNASE3
24ivig459.2IGHE, IL4, IL5
25nedocromil sodium459.2IL5, IL4, RNASE3, IGHE
26bleomycin45 1110.2IFNA1, IL4, IL5
27praziquantel45 1110.2IGHE, IL5, IL4, RNASE3
28azelastine45 1110.2IL5, IL4, IGHE, RNASE3
29montelukast45 51 29 1112.2IL5, IL4, RNASE3, IGHE
30budesonide45 51 1111.2IGHE, RNASE3, IL5, IL4
31fluticasone propionate45 51 61 1112.2RNASE3, IGHE, IL4, IL5
32capsaicin45 2910.1IL5, RNASE3, IGHE
33rantes459.1IL5, IFNA1, RNASE3, IL4
34imatinib45 51 1111.1KIT, IL5, IL4, IFNA1
35murabutide459.0IFNA1, IGHE
36prednisolone45 29 1111.0IL5, IGHE, IFNA1, RNASE3
37pgd2458.7IL5, IGHE, RNASE3, PRG2
38indomethacin45 29 61 1111.5IL5, PRG2, IGHE, IL4
39dexamethasone45 51 29 1111.5IL5, IL4, IGHE, RNASE3, IFNA1
40cycloheximide458.4IGHE, IL4, IFNA1, IL5, KIT
41acetylcholine45 51 29 24 1112.2IGHE, IGES, RNASE3, PRG2
42arginine458.2IL5, IL4, IGHE, PRG2, RNASE3
43vegf458.2IL5, IGHE, IFNA1, KIT, RNASE3, IL4
44cyclosporin a45 29 6110.1IGES, IGHE, IFNA1, IL4, IL5, RNASE3
45actinomycin d458.0KIT, IFNA1, PRG2, IGHE, IL4
46methacholine45 51 119.8PRG2, IL5, RNASE3, IGHE, IGES, IL4
47allergens457.8IL4, PRG2, IL5, IGES, IGHE, RNASE3
48tyrosine457.8IL5, IL4, PRG2, RNASE3, KIT, IFNA1
49retinoic acid45 248.7RNASE3, IL5, KIT, PRG2, IFNA1, IGHE
50histamine45 29 249.4IGES, IL4, PRG2, RNASE3, KIT, IGHE

GO Terms for genes affiliated with Kimura Disease

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16Gene Ontology
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Cellular components related to Kimura Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.3IL5, PRG2, RNASE3, IGHE

Biological processes related to Kimura Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of B cell proliferationGO:0308909.7IL5, IL4
2positive regulation of JAK-STAT cascadeGO:0464279.5KIT, IL5
3positive regulation of tyrosine phosphorylation of Stat5 proteinGO:0425239.3KIT, IL4
4positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.2IL4, IL5, KIT
5immune responseGO:0069558.3IL5, IL4, PRG2, IGHE

Products for genes affiliated with Kimura Disease

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Sources for Kimura Disease

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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