DCM
MCID: DFF001
MIFTS: 34

Diffuse Cutaneous Mastocytosis (DCM) malady

Skin diseases, Immune diseases, Blood diseases, Cancer diseases, Rare diseases categories
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Summaries for Diffuse Cutaneous Mastocytosis

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Sources:
65Wikipedia, 33MalaCards
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Wikipedia:65 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 cutaneous mastocytosis and mastocytosis, and has symptoms including gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia, hepatomegaly/liver enlargement (excluding storage disease) and severe allergic reaction/atopy. An important gene associated with Diffuse Cutaneous Mastocytosis is SLC27A4 (solute carrier family 27 (fatty acid transporter), member 4). Affiliated tissues include skin, liver and bone.

Aliases & Classifications for Diffuse Cutaneous Mastocytosis

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8Disease Ontology, 49Orphanet, 62UMLS, 40NCIt, 35MeSH, 26ICD10 via Orphanet, 63UMLS via Orphanet
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Classifications:



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 62
dcm 49


External Ids:

Disease Ontology8 DOID:3665
NCIt40 C3218
MeSH35 D034701
ICD10 via Orphanet26 Q82.2
UMLS via Orphanet63 C0024901

Related Diseases for Diffuse Cutaneous Mastocytosis

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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
1cutaneous mastocytosis11.0
2mastocytosis11.0
3pseudoxanthomatous diffuse cutaneous mastocytosis10.4
4dilated cardiomyopathy10.4
5bullous diffuse cutaneous mastocytosis10.3
6erythema multiforme10.2
7epidermolysis bullosa10.2
8cutaneous mastocytoma10.2
9microphthalmia10.0
10cataract10.0
11lmna-related dilated cardiomyopathy10.0

Graphical network of diseases related to Diffuse Cutaneous Mastocytosis:



Diseases related to diffuse cutaneous mastocytosis

Symptoms for Diffuse Cutaneous Mastocytosis

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49Orphanet
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Symptoms:

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

Drugs & Therapeutics for Diffuse Cutaneous Mastocytosis

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Sources:
42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Diffuse Cutaneous Mastocytosis

Search NIH Clinical Center for Diffuse Cutaneous Mastocytosis

Genetic Tests for Diffuse Cutaneous Mastocytosis

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Anatomical Context for Diffuse Cutaneous Mastocytosis

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

33
Skin, Liver, Bone, Bone marrow, Monocytes

Animal Models for Diffuse Cutaneous Mastocytosis or affiliated genes

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Publications for Diffuse Cutaneous Mastocytosis

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52PubMed
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Articles related to Diffuse Cutaneous Mastocytosis:

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

Variations for Diffuse Cutaneous Mastocytosis

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Expression for genes affiliated with Diffuse Cutaneous Mastocytosis

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Sources:
2BioGPS, 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

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Compounds for genes affiliated with Diffuse Cutaneous Mastocytosis

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GO Terms for genes affiliated with Diffuse Cutaneous Mastocytosis

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Products for genes affiliated with Diffuse Cutaneous Mastocytosis

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Diffuse Cutaneous Mastocytosis

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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