DCM
MCID: DFF001
MIFTS: 30

Diffuse Cutaneous Mastocytosis (DCM) malady

Skin, Immune, Blood, Cancer categories

Summaries for Diffuse Cutaneous Mastocytosis

Sources:
64Wikipedia, 33MalaCards
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Wikipedia:64 Diffuse cutaneous mastocytosis has diffuse involvement in which the entire integument may be thickened... more...

MalaCards: Diffuse Cutaneous Mastocytosis, also known as diffuse cutaneous maculopapulous mastocytosis, is related to mastocytosis and bullous diffuse cutaneous mastocytosis, and has symptoms including hypotension, dermal/subcutaneous infiltration/induration and hepatomegaly/liver enlargement (excluding storage disease). An important gene associated with Diffuse Cutaneous Mastocytosis is SLC27A4 (solute carrier family 27 (fatty acid transporter), member 4). Affiliated tissues include skin, bone marrow and monocytes.

Aliases & Classifications for Diffuse Cutaneous Mastocytosis

Sources:
8Disease Ontology, 49Orphanet, 61UMLS, 35MeSH, 40NCIt, 26ICD10 via Orphanet, 62UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Skin, Immune, Blood


Characteristics (Orphanet epidemiological data):

49
diffuse cutaneous mastocytosis:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Neonatal/infancy; Age of death: Before age 5


Aliases & Descriptions:

diffuse cutaneous mastocytosis 8 49
diffuse cutaneous maculopapulous mastocytosis 49
mastocytosis, diffuse cutaneous 61
dcm 49


External Ids:

Disease Ontology8 DOID:3665
MeSH35 D034701
NCIt40 C3218
ICD10 via Orphanet26 Q82.2
UMLS via Orphanet62 C0024901

Related Diseases for Diffuse Cutaneous Mastocytosis

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Diffuse Cutaneous Mastocytosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 11)
idRelated DiseaseScoreTop Affiliating Genes
1mastocytosis31.4KIT
2bullous diffuse cutaneous mastocytosis10.6
3pseudoxanthomatous diffuse cutaneous mastocytosis10.5
4mast syndrome10.3
5erythema multiforme10.2
6systemic mastocytosis10.2
7cutaneous mastocytoma10.2
8lmna-related dilated cardiomyopathy10.2
9microphthalmia10.0
10microphthalmia cataract10.0
11myeloid leukemia10.0KIT

Graphical network of diseases related to Diffuse Cutaneous Mastocytosis:



Diseases related to diffuse cutaneous mastocytosis

Clinical Features for Diffuse Cutaneous Mastocytosis

Sources:
49Orphanet
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Symptoms:

49 (show all 16)
  • hypotension
  • dermal/subcutaneous infiltration/induration
  • hepatomegaly/liver enlargement (excluding storage disease)
  • malabsorption/chronic diarrhea/steatorrhea
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • pruritus/itching
  • thick skin/pachydermia/orange skin
  • abnormal pigmentary skin changes/skin pigmentation anomalies
  • urticaria
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • mucosal/cutaneous hemorrhage
  • myeloproliferative syndrome/chronic leukemia
  • mastocytosis
  • severe allergic reaction/atopy
  • hot flushes/sensation of cold

Drugs & Therapeutics for Diffuse Cutaneous Mastocytosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Diffuse Cutaneous Mastocytosis

Anatomical Context for Diffuse Cutaneous Mastocytosis

Sources:
33MalaCards
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MalaCards organs/tissues related to Diffuse Cutaneous Mastocytosis:

33
Skin, Bone marrow, Monocytes

Animal Models for Diffuse Cutaneous Mastocytosis or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Diffuse Cutaneous Mastocytosis

Sources:
51PubMed
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Articles related to Diffuse Cutaneous Mastocytosis:

(show all 44)
idTitleAuthorsYear
1
Generalized bullous eruption after routine vaccination in a child with diffuse cutaneous mastocytosis. (24229828)
2013
2
Diffuse cutaneous bullous mastocytosis in a newborn. (23551376)
2013
3
Diffuse cutaneous mastocytosis: a heterogeneous disease. (23606712)
2013
4
Imatinib mesylate in the treatment of diffuse cutaneous mastocytosis. (23069194)
2013
5
A new germline mutation in KIT associated with diffuse cutaneous mastocytosis in a Chinese family. (24289326)
2013
6
Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature. (22092511)
2012
7
Diffuse cutaneous mastocytosis masquerading as epidermolysis bullosa. (21854415)
2011
8
Diffuse cutaneous mastocytosis: report of a severe case with fatal outcome. (21426873)
2011
9
Oral and topical sodium cromoglicate in the treatment of diffuse cutaneous mastocytosis in an infant. (22693187)
2011
10
Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature. (20678092)
2011
11
Diffuse cutaneous mastocytosis with generalized bullae. (20548889)
2010
12
Clinical aspects of diffuse cutaneous mastocytosis in children: two variants. (19797893)
2009
13
Diffuse cutaneous mastocytosis in fraternal twins. (19200195)
2009
14
Diffuse cutaneous mastocytosis in a newborn calf. (18477335)
2008
15
Systemic mastocytosis with diffuse cutaneous involvement and haematological disease presenting in utero treated unsuccessfully with vincristine. (17983456)
2008
16
Diffuse cutaneous mastocytosis treated with psoralen photochemotherapy: case report and review of the literature. (15656827)
2005
17
A germline mutation in KIT in familial diffuse cutaneous mastocytosis. (15173254)
2004
18
Diffuse cutaneous mastocytosis: pseudoxanthomatous variant. (12126072)
2002
19
Diffuse cutaneous mastocytosis with bone marrow infiltration in a child: a case report. (10886751)
2000
20
Diffuse cutaneous mastocytosis responding to cyproheptadine. (10233642)
1999
21
Diffuse cutaneous mastocytosis with bullae or bullous mastocytosis: a question of semantics. (10627222)
1999
22
Diffuse cutaneous mastocytosis without systemic involvement : Response to topical augmented betamethasone dipropionate under plastic occlusion. (20944388)
1997
23
Ichthyosis congenita type IV: a new case resembling diffuse cutaneous mastocytosis. (9115920)
1997
24
Diffuse cutaneous mastocytosis. Treatment with oral psoralen plus UV-A. (8961869)
1996
25
Diffuse cutaneous mastocytosis: a rare entity. (8415319)
1993
26
Diffuse erythrodermic cutaneous mastocytosis with bone marrow infiltration. (8358101)
1993
27
Continuous release of secretory granule proteoglycans from a cell strain derived from the bone marrow of a patient with diffuse cutaneous mastocytosis. (1728305)
1992
28
Establishment in culture and characterization of a strain with mast cell and monocytic properties from the bone marrow of a child with diffuse cutaneous mastocytosis. (2070067)
1991
29
Diffuse cutaneous mastocytosis mimicking staphylococcal scalded-skin syndrome: report of three cases. (1923984)
1991
30
Diffuse cutaneous mastocytosis, 22 years on. (2337526)
1990
31
The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). (2241201)
1990
32
Photochemotherapy of dominant, diffuse, cutaneous mastocytosis. (2080117)
1990
33
Diffuse cutaneous mastocytosis in a cat. (2238391)
1990
34
Occurrence of platelet-activating factor (PAF) and an endogenous inhibitor of platelet aggregation in diffuse cutaneous mastocytosis. (2805409)
1989
35
Bullous mastocytosis: diffuse cutaneous mastocytosis with extensive blisters mimicking scalded skin syndrome or erythema multiforme. (6387670)
1984
36
Diffuse cutaneous mastocytosis with multiple cutaneous mastocytomas. Report of a case with clinical, histopathological and ultrastructural aspects. (6770887)
1980
37
Diffuse cutaneous mastocytosis: a report of neonatal onset. (94218)
1979
38
Clinico-athological study of a case of diffuse cutaneous mastocytosis. (753755)
1977
39
Diffuse cutaneous mastocytosis in mother and daughter. (935150)
1976
40
Atypical diffuse cutaneous mastocytosis. (5033745)
1972
41
Diffuse cutaneous mastocytosis. (5548477)
1971
42
Diffuse cutaneous mastocytosis. (5710058)
1968
43
DIFFUSE CUTANEOUS MASTOCYTOSIS. (14046151)
1963
44
Diffuse cutaneous mastocytosis. (13822837)
1960

Genetic Variations for Diffuse Cutaneous Mastocytosis

Expression for genes affiliated with Diffuse Cutaneous Mastocytosis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Diffuse Cutaneous Mastocytosis

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Pathways for genes affiliated with Diffuse Cutaneous Mastocytosis

Compounds for genes affiliated with Diffuse Cutaneous Mastocytosis

GO Terms for genes affiliated with Diffuse Cutaneous Mastocytosis

Products for genes affiliated with Diffuse Cutaneous Mastocytosis

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Sources for Diffuse Cutaneous Mastocytosis

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