MCID: URT008
MIFTS: 37

Urticaria Pigmentosa malady

Category: Blood diseases

Aliases & Classifications for Urticaria Pigmentosa

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Aliases & Descriptions for Urticaria Pigmentosa:

Name: Urticaria Pigmentosa 11 13 48 37 66
 
Up/mpcm 11

Classifications:



External Ids:

Disease Ontology11 DOID:12309
ICD1028 Q82.2
MeSH37 D014582
NCIt43 C3433

Summaries for Urticaria Pigmentosa

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Wikipedia:69 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 nodular urticaria pigmentosa and plaque-form urticaria pigmentosa, and has symptoms including welts, macule and darier's sign. An important gene associated with Urticaria Pigmentosa is CMA1 (Chymase 1), 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
1nodular urticaria pigmentosa12.1
2plaque-form urticaria pigmentosa12.0
3typical urticaria pigmentosa12.0
4maculopapular cutaneous mastocytosis11.5
5mast cell disease11.4
6urticaria11.1
7cutaneous mastocytosis10.7
8mastocytosis10.6
9systemic mastocytosis10.4
10filamentary keratitis10.3KIT, KITLG
11movement disease10.3KIT, KITLG
12autosomal recessive non-syndromic intellectual disability10.3KIT, KITLG
13male reproductive system disease10.3FIP1L1, KIT
14congenital disorder of glycosylation, type iq10.3FIP1L1, KIT
15central cloudy dystrophy of francois10.3CMA1, FIP1L1
16nasopharynx carcinoma10.3KIT, KITLG
17asrar facharzt haque syndrome10.2KIT, KITLG
18t-b+ severe combined immunodeficiency due to cd3delta/cd3epsilon/cd3zeta10.2FIP1L1, KIT
19leukemia10.2
20telangiectasia macularis eruptiva perstans10.2
21dentinogenesis imperfecta, shields type ii10.2KIT, KITLG
22multiple spinal canal and spinal cord meningioma10.1KIT, KITLG
23peritoneum cancer10.1IL2RA, KITLG
24restrictive cardiomyopathy10.1KIT, KITLG
25osteoporosis10.1
26juvenile xanthogranuloma10.1
27posteroinferior myocardial infarction10.1CALCA, KIT
28monoclonal mast cell activation syndrome10.0
29pericoronitis10.0CMA1, KIT, KITLG
30cutaneous solitary mastocytoma10.0CMA1, KIT, KITLG
31asthma10.0
32gastroesophageal reflux10.0
33lymphoblastic leukemia10.0
34syringoma10.0
35dermatitis10.0
36indolent systemic mastocytosis10.0
37scabies10.0
38eosinophilia10.0
39homocystinuria10.0KIT, KITLG
40prostate cancer, hereditary, x-linked 19.9KIT, KITLG
41cell type cancer9.9CALCA, KIT
42mastroiacovo de rosa satta syndrome9.9IL2RA, KIT, KITLG
43temporal lobe epilepsy9.9FIP1L1, KIT, KITLG
44loeffler syndrome9.9FIP1L1, KIT, KITLG
45pulmonary coin lesion9.8KIT, VIP
46gastrointestinal stromal tumor9.8
47atopy9.8
48celiac disease9.8
49multiple myeloma9.8
50incontinentia pigmenti9.8

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



Diseases related to urticaria pigmentosa

Symptoms 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
3Cause and Natural Course of Pediatric-Onset MastocytosisCompletedNCT00050193
4Cytokine Production Patterns in Patients With Systemic Mastocytosis Compared With Atopic Dermatitis and Healthy IndividualsCompletedNCT00001760
5Mast Cell Connect: A Registry for Patients With MastocytosisRecruitingNCT02620254
6Study of Factors Regulating Mast Cell ProliferationRecruitingNCT00044122
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:

34
Skin, Bone, Bone marrow, Monocytes, Testes, Thyroid, Smooth muscle

Animal Models for Urticaria Pigmentosa or affiliated genes

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Publications for Urticaria Pigmentosa

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Articles related to Urticaria Pigmentosa:

(show top 50)    (show all 370)
idTitleAuthorsYear
1
Urticaria pigmentosa masquerading as non-accidental injury. (25784750)
2015
2
Telangiectasia macularis eruptiva perstans or highly vascularized urticaria pigmentosa? (25439382)
2014
3
Familial urticaria pigmentosa: report of a family and review of the role of KIT mutations. (22892471)
2013
4
Gastroesophageal reflux disease accompanying urticaria pigmentosa. (22185927)
2011
5
Generalized brownish macules in infancy. Urticaria pigmentosa. (19873965)
2009
6
Intertriginous urticaria pigmentosa. (15604555)
2005
7
Anaphylaxis to insect sting associated with urticaria pigmentosa. (12866320)
2003
8
An infant with urticaria pigmentosa and rickets. (12027094)
2002
9
Reactivated urticaria pigmentosa. (9675535)
1998
10
Determination of the primary structures of human skin chymase and cathepsin G from cutaneous mast cells of urticaria pigmentosa lesions. (8144971)
1994
11
Urticaria pigmentosa: a review of 67 pediatric cases. (8041646)
1994
12
Newer aspects of urticaria pigmentosa. (2357778)
1990
13
Morphological characterization of the somatostatin-immunoreactive dendritic skin cells in urticaria pigmentosa patients by computerized image analysis. (2860719)
1985
14
Immunohistochemical localization of somatostatin-like immunoreactivity in skin lesions from patients with urticaria pigmentosa. (6206641)
1984
15
Decreased urinary histamine metabolite after successful PUVA treatment of urticaria pigmentosa. (7462662)
1981
16
General anesthesia in a child with urticaria pigmentosa. (6106441)
1980
17
Urticaria pigmentosa. Case presentation and discussion. (915191)
1977
18
Urticaria pigmentosa. (1010614)
1976
19
Proceedings: Localized urticaria pigmentosa. (4809211)
1974
20
Urticaria pigmentosa and factitious urticaria. Direct evidence for release of histamine and other smooth muscle-contracting agents in dermographic skin. (4191715)
1970
21
Mastocytosis (urticaria pigmentosa) of skin, stomach, and gut with malabsorption. (6034728)
1967
22
Histamine release from the skin of patients with cutaneous mast cell disease (urticaria pigmentosa). (4162559)
1966
23
Urticaria Pigmentosa with Disseminated Mastocytosis Controlled with Chlorambucil. (19994448)
1965
24
HODGKIN'S DISEASE CONCURRENT WITH URTICARIA PIGMENTOSA. (14043603)
1963
25
Urticaria pigmentosa. Age of onset and prognosis. (13938746)
1962
26
Urticaria pigmentosa, effects of reserpine. (13711436)
1961
27
Histamine formation in urticaria pigmentosa. (13762303)
1961
28
Urticaria pigmentosa with generalized tissue mastocytosis and blood basophilia. (13794652)
1960
29
The roentgen manifestations of urticaria pigmentosa (mastocytosis). (13670326)
1959
30
Histamine and related compounds in urticaria pigmentosa: analyses of tissues having mast-cell infiltration. (13542126)
1958
31
Urticaria pigmentosa; solitary lesions occurring in a mother and her daughter. (13486943)
1958
32
Osseous lesions in urticaria pigmentosa. (13388029)
1957
33
Urticaria pigmentosa; mastocytosis; a consideration of various manifestations. (13457435)
1957
34
The simultaneous occurrence of urticaria pigmentosa and primary systemic amyloidosis: report of a case with autopsy findings. (13429164)
1957
35
Bullous urticaria pigmentosa; summary of literature and report of two cases. (14375388)
1955
36
Urticaria pigmentosa with bone lesions; two cases. (13074214)
1953
37
Urticaria pigmentosa associated with bone lesions; a survey and report of eight cases. (12981299)
1952
38
Urticaria pigmentosa. (15433453)
1950
39
Urticaria pigmentosa of 15 years' duration in a woman aged 41. (14770546)
1950
40
Hydroa puerorum? urticaria pigmentosa. (18135864)
1949
41
URTICARIA pigmentosa]. (21027907)
1946
42
Urticaria Pigmentosa with Unusual Atrophy, in an Adult. (19992636)
1943
43
Urticaria Pigmentosa. (19991647)
1938
44
Urticaria Pigmentosa. (19990687)
1936
45
Urticaria Pigmentosa. (19989426)
1933
46
REPORT OF A CASE OF URTICARIA PIGMENTOSA IN A YOUNG ADULT. (18740130)
1926
47
Urticaria Pigmentosa. (19984305)
1925
48
Urticaria Pigmentosa. (19980941)
1920
49
Case of Urticaria Pigmentosa. (19980755)
1919
50
Urticaria Pigmentosa. (19974197)
1910

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

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1embryonic hemopoiesisGO:003516210.2KIT, KITLG
2ectopic germ cell programmed cell deathGO:003523410.2KIT, KITLG
3regulation of phosphatidylinositol 3-kinase signalingGO:001406610.1KIT, KITLG
4ovarian follicle developmentGO:00015419.8KIT, KITLG
5positive regulation of MAP kinase activityGO:00434069.8KIT, KITLG
6inflammatory responseGO:00069549.2CALCA, IL2RA, KIT
7MAPK cascadeGO:00001659.1IL2RA, KIT, KITLG
8positive regulation of cell proliferationGO:00082848.8KIT, KITLG, VIP

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

idNameGO IDScoreTop Affiliating Genes
1phosphatidylinositol-4,5-bisphosphate 3-kinase activityGO:00469349.7KIT, KITLG
2Ras guanyl-nucleotide exchange factor activityGO:00050889.0IL2RA, KIT, KITLG

Sources for Urticaria Pigmentosa

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet