MCID: URT008
MIFTS: 40

Urticaria Pigmentosa malady

Summaries for Urticaria Pigmentosa

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63Wikipedia, 46OMIM, 32MalaCards
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Wikipedia:63 Urticaria pigmentosa (also known as \"Generalized eruption of cutaneous mastocytosis (childhood type)\"... more...

MalaCards: Urticaria Pigmentosa, also known as up/mpcm, is related to systemic mastocytosis and dermatitis. An important gene associated with Urticaria Pigmentosa is KIT (v-kit Hardy-Zuckerman 4 feline sarcoma viral oncogene homolog), and among its related pathways are Signaling events mediated by Stem cell factor receptor (c-Kit) and Melanocyte Development and Pigmentation. The compounds monochloroacetic acid and ag-1296 have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and bone marrow.

Description from OMIM:46 154800

Aliases & Classifications for Urticaria Pigmentosa

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Sources:
8Disease Ontology, 44Novoseek, 60UMLS, 34MeSH, 46OMIM, 39NCIt, 56SNOMED-CT
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Aliases & Descriptions:

urticaria pigmentosa 8 44 60
up/mpcm 8


External Ids:

Disease Ontology8 DOID:12309
MeSH34 D014582
SNOMED-CT56 205566001, 78745000
OMIM46 154800
NCIt39 C3433

Related Diseases for Urticaria Pigmentosa

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Urticaria Pigmentosa:



Diseases related to urticaria pigmentosa

Clinical Features for Urticaria Pigmentosa

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46OMIM
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Clinical features from OMIM:

154800

Drugs & Therapeutics for Urticaria Pigmentosa

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Urticaria Pigmentosa

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Anatomical Context for Urticaria Pigmentosa

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32MalaCards
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MalaCards organs/tissues related to Urticaria Pigmentosa:

32
Skin, Bone, Bone marrow, Smooth muscle, Thyroid, Testes, Monocytes

Animal Models for Urticaria Pigmentosa or affiliated genes

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Publications for Urticaria Pigmentosa

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50PubMed
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Articles related to Urticaria Pigmentosa:

(show top 50)    (show all 359)
idTitleAuthorsYear
1
Familial urticaria pigmentosa: report of a family and review of the role of KIT mutations. (22892471)
2013
2
Dermatophytosis and papular eosinophilic/mastocytic dermatitis (urticaria pigmentosa-like dermatitis) in three Devon Rex cats. (22736683)
2012
3
Nevoid urticaria pigmentosa along Blaschko's line. (22221017)
2012
4
Anaphylaxis to insect sting associated with urticaria pigmentosa. (12866320)
2003
5
Familial urticaria pigmentosa associated with thrombocytosis as the initial symptom of systemic mastocytosis and Down's syndrome. (14761147)
2003
6
Severe pruritus in a patient with urticaria pigmentosa treated with topical 5% urea and 3% polidocanol cream. (14522633)
2003
7
Urticaria pigmentosa associated with Wilms tumor. (11576405)
2001
8
Lack of c-kit mutation in familial urticaria pigmentosa. (10354021)
1999
9
Short- and long-term effectiveness of oral and bath PUVA therapy in urticaria pigmentosa and systemic mastocytosis. (9267734)
1997
10
Dermacase. Urticaria pigmentosa. (7647621)
1995
11
Eruptive syringoma mimicking urticaria pigmentosa. A case report. (8103261)
1993
12
Histamine release from skin mast cells and basophils in patients with urticaria pigmentosa. (1690493)
1990
13
Familial urticaria pigmentosa. (2383167)
1990
14
Bone marrow findings in adult patients with urticaria pigmentosa. (3162249)
1988
15
Preleukemia and urticaria pigmentosa followed by acute myelomonoblastic leukemia. (3856421)
1985
16
A cutaneous manifestation of acute lymphoblastic leukaemia mimicking urticaria pigmentosa. (6588373)
1984
17
Phagocytosis by mast cells in urticaria pigmentosa. (6208713)
1984
18
The treatment of urticaria pigmentosa. (6809372)
1982
19
Hematologic malignancies occurring in patients with urticaria pigmentosa. (6957422)
1982
20
Calcification of cultured urticaria pigmentosa skin. An electron optical study of the calcium-apatite deposition. (7426398)
1980
21
Urticaria pigmentosa. Case presentation and discussion. (915191)
1977
22
Generalized mastocytosis in a foal, resembling urticaria pigmentosa of man. (1089100)
1975
23
Congenital macular urticaria pigmentosa complicated by massive nodular mastocytosis with systemic involvement. (4648806)
1972
24
Urticaria pigmentosa and factitious urticaria. Direct evidence for release of histamine and other smooth muscle-contracting agents in dermographic skin. (4191715)
1970
25
Solitary mast cell tumor. Progression to disseminated urticaria pigmentosa in a negro infant. (4973469)
1969
26
Histamine release from the skin of patients with cutaneous mast cell disease (urticaria pigmentosa). (4162559)
1966
27
URINARY EXCRETION OF ACID MUCOPOLYSACCHARIDES IN TWELVE CASES OF MASTOCYTOSIS (URTICARIA PIGMENTOSA). (14209458)
1964
28
ELECTRONMICROSCOPIC OBSERVATIONS OF MAST CELLS IN THE LESIONS OF URTICARIA PIGMENTOSA. (14071304)
1963
29
Solitary mastocytosis (urticaria pigmentosa). (14452118)
1961
30
Histaminuria in urticaria pigmentosa. A clinical study and review of recent literature with definition of the mastocytosis syndrome. (13721777)
1961
31
A case of widespread urticaria pigmentosa with episodes of flushing. (13842513)
1960
32
Urticaria pigmentosa; report of a case with autopsy examination. (13648961)
1959
33
Urticaria pigmentosa with systemic mast-cell disease. (13410111)
1957
34
Release of histamine in urticaria pigmentosa. (13417625)
1957
35
Generalized osteosclerosis in urticaria pigmentosa, the radiologic aspect. (13372383)
1956
36
Two cases of urticaria pigmentosa]. (14362570)
1954
37
Urticaria pigmentosa with bone lesions. (13123742)
1953
38
Urticaria pigmentosa: report of a case. (14873115)
1951
39
Urticaria pigmentosa; report of a case of the bullous variety in a newborn infant. (14898406)
1951
40
Urticaria pigmentosa; a report of a case with autopsy. (18149230)
1949
41
Urticaria pigmentosa with bullae. (20292644)
1947
42
Urticaria pigmentosa. (21000217)
1946
43
Urticaria Pigmentosa with Unusual Atrophy, in an Adult. (19992636)
1943
44
Urticaria Pigmentosa. (19992159)
1939
45
Urticaria Pigmentosa treated with Grenz-rays. (19991906)
1939
46
Urticaria Pigmentosa. (19989426)
1933
47
Urticaria Pigmentosa. (19984305)
1925
48
Case for Diagnosis (? Urticaria Pigmentosa). (19983593)
1924
49
Urticaria Pigmentosa in an Adult. (19982867)
1923
50
Urticaria pigmentosa in a Girl aged 10. (19973550)
1909

Genetic Variations for Urticaria Pigmentosa

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Expression for genes affiliated with Urticaria Pigmentosa

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Urticaria Pigmentosa

Search GEO for disease gene expression data for Urticaria Pigmentosa.

Pathways for genes affiliated with Urticaria Pigmentosa

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37NCBI BioSystems Database, 51QIAGEN, 53Reactome, 29KEGG, 4Cell Signaling Technology
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Pathways related to Urticaria Pigmentosa according to GeneCards/GeneDecks:

(show all 19)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
9.5KIT, KITLG
29.5KIT, KITLG
39.5KIT, KITLG
49.5KITLG, KIT
5
Hide members
9.0IL2RA, KITLG, KIT
6
Hide members
9.0IL2RA, KITLG, KIT
7
Hide members
9.0IL2RA, KITLG, KIT
8
Hide members
9.0KIT, KITLG, IL2RA
9
Hide members
9.0IL2RA, KITLG, KIT
10
Hide members
9.0KIT, KITLG, IL2RA
11
Hide members
9.0KIT, KITLG, IL2RA
12
Hide members
9.0IL2RA, KITLG, KIT
139.0IL2RA, KITLG, KIT
149.0KIT, KITLG, IL2RA
159.0KIT, KITLG, IL2RA
169.0KIT, KITLG, IL2RA
17
Hide members
9.0KIT, KITLG, IL2RA
18
Hide members
9.0IL2RA, KITLG, KIT
19
Hide members
8.9KIT, KITLG, CMA1

Compounds for genes affiliated with Urticaria Pigmentosa

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44Novoseek, 49PharmGKB, 59Tocris Bioscience, 2BitterDB, 28IUPHAR, 11DrugBank, 24HMDB
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Compounds related to Urticaria Pigmentosa according to GeneCards/GeneDecks:

(show all 26)
idCompoundScoreTop Affiliating Genes
1monochloroacetic acid449.9KIT, KITLG
2ag-1296449.8KIT, KITLG
3su5416449.8KITLG, KIT
4toluidine449.8KITLG, KIT
5rhodamine 12344 4910.8KITLG, KIT
62-chlorodeoxyadenosine449.8KIT, IL2RA
7as 10159 4410.7IL2RA, KITLG
8methylcellulose449.7KIT, KITLG
9chloroquine44 2 49 28 1113.6IL2RA, KIT
10hydroxyurea44 49 1111.6KIT, KITLG
11cytarabine44 49 1111.5KIT, KITLG
12vincristine44 49 1111.4IL2RA, KIT
13gp 130449.3IL2RA, KITLG, KIT
14methotrexate44 49 1111.3KIT, KITLG, IL2RA
155fluorouracil449.3IL2RA, KITLG, KIT
16paclitaxel44 49 1111.3KIT, KITLG, IL2RA
17rapamycin449.3IL2RA, KITLG, KIT
18doxorubicin44 49 1111.2KIT, KITLG, IL2RA
19thymidine44 2410.2IL2RA, KITLG, KIT
20testosterone44 59 11 2412.2KIT, KITLG, IL2RA
21steroid449.1KIT, KITLG, IL2RA
22heparin44 28 11 2412.0KITLG, CMA1, IL2RA
23phosphatidylinositol449.0KIT, KITLG, IL2RA
24histamine44 28 2410.7KIT, KITLG, CMA1, IL2RA
25paraffin448.7KIT, KITLG, CMA1, IL2RA
26vegf448.7KIT, KITLG, IL2RA

GO Terms for genes affiliated with Urticaria Pigmentosa

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16Gene Ontology
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Biological processes related to Urticaria Pigmentosa according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1germ cell programmed cell deathGO:0352349.5KIT, KITLG
2male gonad developmentGO:0085849.5KIT, KITLG
3embryonic hemopoiesisGO:0351629.4KIT, KITLG
4hemopoiesisGO:0300979.2KIT, KITLG

Products for genes affiliated with Urticaria Pigmentosa

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  • Antibodies
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Sources for Urticaria Pigmentosa

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet