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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Wikipedia:65 Diffuse cutaneous mastocytosis has diffuse involvement in which the entire integument may be thickened... more...

MalaCards based summary: Diffuse Cutaneous Mastocytosis, also known as diffuse cutaneous maculopapulous mastocytosis, is related to cutaneous mastocytosis and mastocytosis, and has symptoms including erythema/erythematous lesions/erythroderma/polymorphous erythema, vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment and dermal/subcutaneous infiltration/induration. 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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Sources:
8Disease Ontology, 48Orphanet, 62UMLS, 34MeSH, 39NCIt, 26ICD10 via Orphanet, 63UMLS via Orphanet
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Diffuse Cutaneous Mastocytosis, Aliases & Descriptions:

Name: Diffuse Cutaneous Mastocytosis 8 48
Diffuse Cutaneous Maculopapulous Mastocytosis 48
 
Mastocytosis, Diffuse Cutaneous 62
Dcm 48


Classifications:



Characteristics (Orphanet epidemiological data):

48
diffuse cutaneous mastocytosis:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Neonatal/infancy; Age of death: Before age 5


External Ids:

Disease Ontology8 DOID:3665
MeSH34 D034701
NCIt39 C3218
ICD10 via Orphanet26 Q82.2
UMLS via Orphanet63 C0024901

Related Diseases for Diffuse Cutaneous Mastocytosis

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

idRelated DiseaseScoreTop Affiliating Genes
1cutaneous mastocytosis11.0
2mastocytosis11.0
3pseudoxanthomatous diffuse cutaneous mastocytosis10.4
4dilated cardiomyopathy10.4
5bullous diffuse cutaneous mastocytosis10.4
6erythema multiforme10.2
7cutaneous mastocytoma10.2
8cataract10.0
9microphthalmia10.0
10lmna-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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Symptoms:

48 (show all 16)
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • 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
  • mucosal/cutaneous hemorrhage
  • hot flushes/sensation of cold
  • malabsorption/chronic diarrhea/steatorrhea
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • hepatomegaly/liver enlargement (excluding storage disease)
  • severe allergic reaction/atopy
  • myeloproliferative syndrome/chronic leukemia

HPO human phenotypes related to Diffuse Cutaneous Mastocytosis:

(show all 14)
id Description Frequency HPO Source Accession
1 abnormal blistering of the skin hallmark (90%) HP:0008066
2 mastocytosis hallmark (90%) HP:0100495
3 pruritus typical (50%) HP:0000989
4 abnormality of skin pigmentation typical (50%) HP:0001000
5 urticaria typical (50%) HP:0001025
6 thickened skin typical (50%) HP:0001072
7 subcutaneous hemorrhage typical (50%) HP:0001933
8 hypotension typical (50%) HP:0002615
9 impaired temperature sensation typical (50%) HP:0010829
10 leukemia occasional (7.5%) HP:0001909
11 malabsorption occasional (7.5%) HP:0002024
12 gastrointestinal hemorrhage occasional (7.5%) HP:0002239
13 hepatomegaly occasional (7.5%) HP:0002240
14 immunologic hypersensitivity occasional (7.5%) HP:0100326

Drugs & Therapeutics for Diffuse Cutaneous Mastocytosis

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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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MalaCards organs/tissues related to Diffuse Cutaneous Mastocytosis:

32
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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Articles related to Diffuse Cutaneous Mastocytosis:

(show all 39)
idTitleAuthorsYear
1
Diffuse cutaneous mastocytosis, bullous variant, presenting in a six-month-old infant. (24811027)
2014
2
Generalized bullous eruption after routine vaccination in a child with diffuse cutaneous mastocytosis. (24229828)
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: 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 without systemic involvement : Response to topical augmented betamethasone dipropionate under plastic occlusion. (20944388)
1997
20
Ichthyosis congenita type IV: a new case resembling diffuse cutaneous mastocytosis. (9115920)
1997
21
Diffuse cutaneous mastocytosis. Treatment with oral psoralen plus UV-A. (8961869)
1996
22
Diffuse cutaneous mastocytosis: a rare entity. (8415319)
1993
23
Continuous release of secretory granule proteoglycans from a cell strain derived from the bone marrow of a patient with diffuse cutaneous mastocytosis. (1728305)
1992
24
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
25
Diffuse cutaneous mastocytosis, 22 years on. (2337526)
1990
26
The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). (2241201)
1990
27
Photochemotherapy of dominant, diffuse, cutaneous mastocytosis. (2080117)
1990
28
Diffuse cutaneous mastocytosis in a cat. (2238391)
1990
29
Occurrence of platelet-activating factor (PAF) and an endogenous inhibitor of platelet aggregation in diffuse cutaneous mastocytosis. (2805409)
1989
30
Bullous mastocytosis: diffuse cutaneous mastocytosis with extensive blisters mimicking scalded skin syndrome or erythema multiforme. (6387670)
1984
31
Diffuse cutaneous mastocytosis with multiple cutaneous mastocytomas. Report of a case with clinical, histopathological and ultrastructural aspects. (6770887)
1980
32
Diffuse cutaneous mastocytosis: a report of neonatal onset. (94218)
1979
33
Clinico-athological study of a case of diffuse cutaneous mastocytosis. (753755)
1977
34
Diffuse cutaneous mastocytosis in mother and daughter. (935150)
1976
35
Atypical diffuse cutaneous mastocytosis. (5033745)
1972
36
Diffuse cutaneous mastocytosis. (5548477)
1971
37
Diffuse cutaneous mastocytosis. (5710058)
1968
38
DIFFUSE CUTANEOUS MASTOCYTOSIS. (14046151)
1963
39
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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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
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Sources for Diffuse Cutaneous Mastocytosis

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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet