MCID: URT008
MIFTS: 38

Urticaria Pigmentosa malady

Category: Blood diseases

Aliases & Classifications for Urticaria Pigmentosa

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Aliases & Descriptions for Urticaria Pigmentosa:

Name: Urticaria Pigmentosa 11 50 39 13 68
 
Up/mpcm 11

Classifications:



External Ids:

Disease Ontology11 DOID:12309
ICD1030 Q82.2
MeSH39 D014582
NCIt45 C3433

Summaries for Urticaria Pigmentosa

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Wikipedia:71 Urticaria pigmentosa (also known as \"Generalized eruption of cutaneous mastocytosis (childhood type)\"... more...

MalaCards based summary: Urticaria Pigmentosa, also known as up/mpcm, is related to asthma and nodular urticaria pigmentosa, and has symptoms including welts, macule and darier's sign. An important gene associated with Urticaria Pigmentosa is KIT (KIT Proto-Oncogene Receptor Tyrosine Kinase), and among its related pathways are C-MYB transcription factor network and Regulation of KIT signaling. Affiliated tissues include skin, bone and bone marrow.

Related Diseases for Urticaria Pigmentosa

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Diseases related to Urticaria Pigmentosa via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 96)
idRelated DiseaseScoreTop Affiliating Genes
1asthma28.3IL2RA, KITLG, TAC1, VIP
2nodular urticaria pigmentosa12.0
3plaque-form urticaria pigmentosa11.9
4typical urticaria pigmentosa11.9
5mast cell disease11.4
6maculopapular cutaneous mastocytosis11.0
7urticaria11.0
8mastocytosis10.5
9filamentary keratitis10.3KIT, KITLG
10movement disease10.3KIT, KITLG
11autosomal recessive non-syndromic intellectual disability10.3KIT, KITLG
12systemic mastocytosis10.2
13asrar facharzt haque syndrome10.2KIT, KITLG
14nonsyndromic congenital nail disorder 210.2KIT, TAC1
15temporal lobe epilepsy10.2KIT, KITLG
16peritoneum cancer10.1IL2RA, KITLG
17frontal sinus squamous cell carcinoma10.1KIT, TAC1
18dentinogenesis imperfecta, shields type ii10.1KIT, KITLG
19multiple spinal canal and spinal cord meningioma10.1KIT, KITLG
20posteroinferior myocardial infarction10.1CALCA, KIT
21leukemia10.1
22cutaneous mastocytosis10.1
23telangiectasia macularis eruptiva perstans10.1
24calpainopathy10.1CALCA, TAC1
25restrictive cardiomyopathy10.1KIT, KITLG
26loeffler syndrome10.0KIT, KITLG
27constrictive pericarditis10.0CALCA, TAC1
28diffuse cutaneous mastocytosis10.0CMA1, KIT, KITLG
29pericoronitis10.0CMA1, KIT, KITLG
30cutaneous solitary mastocytoma10.0CMA1, KIT, KITLG
31duodenal obstruction10.0CMA1, KIT, KITLG
32tracheal stenosis10.0CALCA, TAC1
33thrombocythemia 310.0KIT, KITLG
34osteoporosis10.0
35juvenile xanthogranuloma10.0
36intussusception9.9CALCA, TAC1
37focal hand dystonia9.9TAC1, VIP
38pulmonary coin lesion9.9KIT, VIP
39gastroesophageal reflux9.9
40lymphoblastic leukemia9.9
41syringoma9.9
42dermatitis9.9
43indolent systemic mastocytosis9.9
44scabies9.9
45eosinophilia9.9
46mastroiacovo de rosa satta syndrome9.9IL2RA, KIT, KITLG
47cell type cancer9.8CALCA, KIT
48liver leiomyoma9.8KIT, VIP
49calciphylaxis9.8CALCA, VIP
50gastrinoma9.8CALCA, VIP

Graphical network of the top 20 diseases related to Urticaria Pigmentosa:



Diseases related to urticaria pigmentosa

Symptoms & Phenotypes for Urticaria Pigmentosa

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UMLS symptoms related to Urticaria Pigmentosa:


welts, macule, darier's sign, instillation site urticaria

Drugs & Therapeutics for Urticaria Pigmentosa

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Interventional clinical trials:

idNameStatusNCT IDPhase
1Ultraviolet (UVA and UVB) Light Therapy in the Treatment of Inflammatory Skin ConditionsTerminatedNCT00129415Phase 1, Phase 2
2Natural History of Urticaria Pigmentosa in ChildrenCompletedNCT00467792
3Cytokine Production Patterns in Patients With Systemic Mastocytosis Compared With Atopic Dermatitis and Healthy IndividualsCompletedNCT00001760
4Cause and Natural Course of Pediatric-Onset MastocytosisCompletedNCT00050193
5Study of Factors Regulating Mast Cell ProliferationRecruitingNCT00044122
6Mast Cell Connect: A Registry for Patients With MastocytosisRecruitingNCT02620254
7Cutaneous Mastocytosis in Children: Analysis of Somatic and Germline MutationsNot yet recruitingNCT02761473

Search NIH Clinical Center for Urticaria Pigmentosa


Cochrane evidence based reviews: urticaria pigmentosa

Genetic Tests for Urticaria Pigmentosa

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Anatomical Context for Urticaria Pigmentosa

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MalaCards organs/tissues related to Urticaria Pigmentosa:

36
Skin, Bone, Bone marrow, Monocytes, Testes, Thyroid, Smooth muscle

Publications for Urticaria Pigmentosa

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Articles related to Urticaria Pigmentosa:

(show top 50)    (show all 370)
idTitleAuthorsYear
1
General Anesthesia in a Patient With Urticaria Pigmentosa Referred for Electroconvulsive Therapy. (26934274)
2016
2
Urticaria pigmentosa masquerading as non-accidental injury. (25784750)
2015
3
Urticaria pigmentosa-like disease in a dog. (25750443)
2015
4
Urticaria pigmentosa. (26752589)
2015
5
Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine. (25515765)
2015
6
NKp46 regulates the production of serine proteases and IL-22 in human mast cells in urticaria pigmentosa. (25940096)
2015
7
Telangiectasia macularis eruptiva perstans or highly vascularized urticaria pigmentosa? (25439382)
2014
8
Urticaria pigmentosa complicated with esophageal eosinophilia. (25437994)
2014
9
A segmental rash in a young male: a quiz. Naevoid urticaria pigmentosa. (23828224)
2014
10
A 1-year-old boy with persistent, generalized eruption. Urticaria pigmentosa. (24549082)
2014
11
Familial urticaria pigmentosa: report of a family and review of the role of KIT mutations. (22892471)
2013
12
Dermatoscopic findings of urticaria pigmentosa. (24474113)
2013
13
Urticaria pigmentosa in a female patient with celiac disease: response to a gluten-free diet. (22465751)
2013
14
Response of urticaria pigmentosa to cladribine in a patient with systemic mastocytosis. (23205635)
2013
15
Cutaneous mastocytosis: a case of bullous urticaria pigmentosa. (23513553)
2013
16
Acute interstitial nephritis secondary to long-term use of cetirizine for the treatment of urticaria pigmentosa. (23083330)
2013
17
Dermatophytosis and papular eosinophilic/mastocytic dermatitis (urticaria pigmentosa-like dermatitis) in three Devon Rex cats. (22736683)
2012
18
Nevoid urticaria pigmentosa along Blaschko's line. (22221017)
2012
19
Gastroesophageal reflux disease accompanying urticaria pigmentosa. (22185927)
2011
20
Cutaneous mastocytosis in twins: multiple mastocytomas and urticaria pigmentosa in two pairs of monozygotic twins. (21385209)
2011
21
Urticaria pigmentosa after treatment with TNF antagonists: a case report. (22184778)
2011
22
Generalized brownish macules in infancy. Urticaria pigmentosa. (19873965)
2009
23
Megalocornea-urticaria pigmentosa syndrome--a new syndrome? (19706342)
2009
24
Urticaria pigmentosa associated with acute stress and lesional skin mast-cell expression of CRF-R1. (19094125)
2009
25
Picture of the month. Urticaria pigmentosa. (18391148)
2008
26
What is your diagnosis? Cutaneous mastocytosis (urticaria pigmentosa). (18543588)
2008
27
Urticaria pigmentosa. A case report. (18709295)
2008
28
Mastocytosis with urticaria pigmentosa and osteoporosis. (19061601)
2008
29
CD25 expression on cutaneous mast cells from adult patients presenting with urticaria pigmentosa is predictive of systemic mastocytosis. (18162781)
2008
30
Unusual cutaneous findings of urticaria pigmentosa and telangiectasia macularis eruptiva perstans associated with marked myelofibrosis. (17040445)
2006
31
Anesthetic management of a labouring parturient with urticaria pigmentosa. (16575037)
2006
32
Urticaria pigmentosa and mastocytosis: the role of immunophenotyping in diagnosis and determining response to treatment. (16822380)
2006
33
Intertriginous urticaria pigmentosa. (15604555)
2005
34
The cosmetic treatment of urticaria pigmentosa with Nd:YAG laser at 532 nanometers. (17166203)
2005
35
Nodular scabies mimicking urticaria pigmentosa in an infant. (16045712)
2005
36
Localized urticaria pigmentosa triggered by mesotherapy. (15539904)
2004
37
Papular eosinophilic/mastocytic dermatitis (feline urticaria pigmentosa) in Devon Rex cats: A distinct disease entity or a histopathological reaction pattern? (15305933)
2004
38
Urticaria pigmentosa. (15120152)
2004
39
Anaphylaxis to insect sting associated with urticaria pigmentosa. (12866320)
2003
40
Severe pruritus in a patient with urticaria pigmentosa treated with topical 5% urea and 3% polidocanol cream. (14522633)
2003
41
Urticaria pigmentosa localized on radiation field. (12948928)
2003
42
Evolution of urticaria pigmentosa into indolent systemic mastocytosis: abnormal immunophenotype of mast cells without evidence of c-kit mutation ASP-816-VAL. (12688351)
2003
43
Familial urticaria pigmentosa associated with thrombocytosis as the initial symptom of systemic mastocytosis and Down's syndrome. (14761147)
2003
44
Medium- versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study. (14512916)
2003
45
Association of urticaria pigmentosa with cafAc-au-lait spots, neurofibromas and neurofibroma-like neoplasms: a mere coincidence? (12771469)
2003
46
An infant with urticaria pigmentosa and rickets. (12027094)
2002
47
Infantile fibrosarcoma associated with urticaria pigmentosa. (12186369)
2002
48
Mast cells with bilobed or multilobed nuclei in a nodular lesion of a patient with urticaria pigmentosa. (12454601)
2002
49
Regression of urticaria pigmentosa in adult patients with systemic mastocytosis: correlation with clinical patterns of disease. (12056960)
2002
50
Scabies incognito presenting as urticaria pigmentosa in an infant. (12383096)
2002

Variations for Urticaria Pigmentosa

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Expression for genes affiliated with Urticaria Pigmentosa

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Search GEO for disease gene expression data for Urticaria Pigmentosa.

Pathways for genes affiliated with Urticaria Pigmentosa

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GO Terms for genes affiliated with Urticaria Pigmentosa

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Cellular components related to Urticaria Pigmentosa according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1neuronal cell bodyGO:00430258.5CALCA, TAC1, VIP
2extracellular regionGO:00055768.3CALCA, CMA1, KITLG, TAC1, VIP

Biological processes related to Urticaria Pigmentosa according to GeneCards Suite gene sharing:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1ectopic germ cell programmed cell deathGO:003523410.3KIT, KITLG
2embryonic hemopoiesisGO:003516210.3KIT, KITLG
3ovarian follicle developmentGO:000154110.2KIT, KITLG
4positive regulation of MAP kinase activityGO:004340610.2KIT, KITLG
5cellular response to nerve growth factor stimulusGO:199009010.0CALCA, TAC1
6regulation of phosphatidylinositol 3-kinase signalingGO:001406610.0KIT, KITLG
7positive regulation of ossificationGO:00457789.9CALCA, TAC1
8MAPK cascadeGO:00001659.7IL2RA, KIT, KITLG
9response to painGO:00482659.3CALCA, TAC1
10inflammatory responseGO:00069549.1CALCA, IL2RA, KIT, TAC1

Molecular functions related to Urticaria Pigmentosa according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1phosphatidylinositol-4,5-bisphosphate 3-kinase activityGO:004693410.2KIT, KITLG
2Ras guanyl-nucleotide exchange factor activityGO:00050889.1IL2RA, KIT, KITLG

Sources for Urticaria Pigmentosa

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet