MCID: DRM007
MIFTS: 66

Dermatitis Herpetiformis malady

Skin diseases category

Summaries for Dermatitis Herpetiformis

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63Wikipedia, 46OMIM, 32MalaCards
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Wikipedia:63 Dermatitis herpetiformis (DH), or Duhring\'s disease, is a chronic blistering skin condition,... more...

MalaCards: Dermatitis Herpetiformis, also known as dermatosis herpetiformis, is related to dermatitis and celiac disease, and has symptoms including bone pain, cutaneous edema and erythema/erythematous lesions/erythroderma/polymorphous erythema. An important gene associated with Dermatitis Herpetiformis is CD79A (CD79a molecule, immunoglobulin-associated alpha), and among its related pathways are Matrix Metalloproteinases and Degradation of the extracellular matrix. The drugs dapsone and sulfapyridine pwdr and the compounds glutamate and gliadin have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and t cells, and related mouse phenotypes are respiratory system and renal/urinary system.

Description from OMIM:46 601230

Aliases & Classifications for Dermatitis Herpetiformis

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Sources:
8Disease Ontology, 10DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 60UMLS, 56SNOMED-CT, 27ICD9CM, 39NCIt, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Skin diseases


Characteristics (Orphanet epidemiological data):

48
dermatitis herpetiformis:
Inheritance: Sporadic; Prevalence: 1-5/10000; Age of onset: Variable


Aliases & Descriptions:

dermatitis herpetiformis 8 10 44 48 46 60
dermatosis herpetiformis 8
durhing-brocq disease 48
duhring's disease 8


External Ids:

Disease Ontology8 DOID:8505
ICD9CM27 694.0
NCIt39 C26742
MeSH34 D003874
OMIM46 601230
MESH via Orphanet35 D003874
ICD10 via Orphanet26 L13.0
SNOMED-CT via Orphanet57 111196000
UMLS via Orphanet61 C0011608
ICD1025 L13.0

Related Diseases for Dermatitis Herpetiformis

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Sources:
17GeneCards, 18GeneDecks
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Diseases in the Dermatitis Herpetiformis family:

Juvenile Dermatitis Herpetiformis Dermatitis Herpetiformis Familial

Diseases related to Dermatitis Herpetiformis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 176)
idRelated DiseaseScoreTop Affiliating Genes
1dermatitis32.1CD79A, CCL11
2celiac disease31.2HLA-DQB1, TRB, MMP12, TGM2, CD79A
3bullous pemphigoid31.0CCL11, CD79A, PLAU, HLA-DQB1
4ulcerative colitis30.6MMP7, MMP3, CCL11, CD79A
5psoriasis30.5TGM3, TRB, TGM1, CCL11
6skin disease30.5TGM3, CCL11, TRB, CD79A, TGM1
7arthritis30.4FBN1, MMP3, TRB, MMP12, IL2RB
8purpura30.3CD79A
9pemphigus30.3PLAU, CD79A
10diabetes mellitus30.3MMP3, CD79A, HLA-DQB1
11systemic lupus erythematosus30.3IL2RB, CD79A, TRB, MMP3, FBN1, HLA-DQB1
12rheumatoid arthritis30.3MMP12, TRB, CD79A, IL2RB, MMP3, HLA-DQB1
13myasthenia gravis30.1HLA-DQB1
14stevens-johnson syndrome30.1TGM1
15ankylosing spondylitis30.1CD79A
16hepatitis c30.1MMP7
17hepatitis30.1CCL11, IL2RB
18sarcoidosis30.1IL2RB, CCL11, TRB, HLA-DQB1
19gastroenteritis30.1CD79A
20leukemia30.1CD79A, CCL11, TRB, IL2RB
21lung cancer30.1TRB, PLAU, MMP7, MMP3
22juvenile dermatitis herpetiformis10.7
23lupus erythematosus10.5
24vitiligo10.4
25thyroiditis10.4
26herpes gestationis10.4
27gastritis10.4
28intestinal disease10.4
29dermatitis herpetiformis familial10.4
30duodenitis10.3
31sclerosing cholangitis10.3
32cholangitis10.3
33epidermolysis bullosa10.3
34urticaria10.3
35impetigo herpetiformis10.2
36subcorneal pustular dermatosis10.2
37atrophic gastritis10.2
38primary biliary cirrhosis10.2
39protein-losing enteropathy10.2
40stiff-person syndrome10.2
41anaplastic large cell lymphoma10.2
42cutaneous t cell lymphoma10.2
43glomerulonephritis10.2
44impetigo10.2
45laryngitis10.2
46pancreatitis10.2
47duhring brocq disease10.2
48deficiency anemia10.1
49membranous glomerulonephritis10.1
50relapsing polychondritis10.1

Graphical network of the top 20 diseases related to Dermatitis Herpetiformis:



Diseases related to dermatitis herpetiformis

Clinical Features for Dermatitis Herpetiformis

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Sources:
46OMIM, 48Orphanet
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Clinical features from OMIM:

601230

Clinical synopsis from OMIM:

601230

Symptoms:

48 (show all 15)
  • bone pain
  • cutaneous edema
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • macules
  • follicular/erythematous/edematous papules/milium
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • negative nikolski's sign
  • pruritus/itching
  • malabsorption/chronic diarrhea/steatorrhea
  • mutiple fractures/bone fragility
  • autoimmunity/autoimmune reaction/autoantibodies
  • microcytic anemia
  • eczema
  • lichen
  • urticaria

Drugs & Therapeutics for Dermatitis Herpetiformis

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

Search CenterWatch for Dermatitis Herpetiformis

Drug clinical trials:

Search ClinicalTrials for Dermatitis Herpetiformis

Search NIH Clinical Center for Dermatitis Herpetiformis

Search CenterWatch for Dermatitis Herpetiformis

Inferred drug relations via UMLS60/NDF-RT40:

Genetic Tests for Dermatitis Herpetiformis

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Anatomical Context for Dermatitis Herpetiformis

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Sources:
32MalaCards
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MalaCards organs/tissues related to Dermatitis Herpetiformis:

32
Skin, Bone, T cells, Thyroid, Small intestine, Testes, Endothelial, Pituitary, Heart, Colon, Liver, Lung, Tonsil

Animal Models for Dermatitis Herpetiformis or affiliated genes

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Sources:
36MGI
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MGI Mouse Phenotypes related to Dermatitis Herpetiformis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053889.8CCL11, HLA-DQB1, EDNRB, FBN1, TRB, MMP12
2MP:00053679.7EDNRB, FBN1, TRB, CD79A, TGM2, HLA-DQB1
3MP:00107719.7EDNRB, FBN1, TRB, MMP12, TGM1
4MP:00053859.5HLA-DQB1, EDNRB, FBN1, TRB, MMP12, MMP7
5MP:00053769.4CD79A, FBN1, EDNRB, HLA-DQB1, MMP3, MMP12
6MP:00053849.4HLA-DQB1, EDNRB, FBN1, TRB, CD79A, IL2RB
7MP:00053979.3CCL11, HLA-DQB1, FBN1, TRB, TRAT1, CD79A
8MP:00036319.1HLA-DQB1, EDNRB, FBN1, TRB, CD79A, MMP3
9MP:00053879.0TGM2, CCL11, HLA-DQB1, EDNRB, FBN1, TRB

Publications for Dermatitis Herpetiformis

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Sources:
50PubMed
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Articles related to Dermatitis Herpetiformis:

(show top 50)    (show all 537)
idTitleAuthorsYear
1
Acral purpura as leading clinical manifestation of dermatitis herpetiformis: report of two adult cases with a review of the literature. (23970137)
2013
2
Long-term combination therapy of ustekinumab and dapsone in a patient with psoriasis and dermatitis herpetiformis Duhring. (22784106)
2012
3
Reduced mortality in dermatitis herpetiformis: a population-based study of 476 patients. (22708883)
2012
4
Recent advances in dermatitis herpetiformis. (22811741)
2012
5
Dermatitis herpetiformis. Part I. Epidemiology, pathogenesis, and clinical presentation. (21571167)
2011
6
A case of stiff-person syndrome, type 1 diabetes, celiac disease and dermatitis herpetiformis. (19150172)
2009
7
Transglutaminase autoantibodies in dermatitis herpetiformis and celiac sprue. (17762854)
2008
8
Increased frequency of Ig heavy-chain HS1,2-A enhancer *2 allele in dermatitis herpetiformis, plaque psoriasis, and psoriatic arthritis. (18323783)
2008
9
Dermatitis herpetiformis in a patient with ulcerative colitis. (18183600)
2008
10
Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives. (15656805)
2005
11
Dermatitis herpetiformis with flame figures mimicking an arthropod bite. (12775996)
2003
12
Genetic dissection between coeliac disease and dermatitis herpetiformis in sib pairs. (12485471)
2002
13
Characterization of a subgroup of patients with dermatitis herpetiformis with nonclassical histologic features. (12142608)
2002
14
Dermatitis herpetiformis: should direct immunofluorescence be the only diagnostic criterion? (12220281)
2002
15
Parallel expression of macrophage metalloelastase (MMP-12) in duodenal and skin lesions of patients with dermatitis herpetiformis. (11247893)
2001
16
Expression of interleukin-4 and interferon-gamma in the small bowel of patients with dermatitis herpetiformis and isolated gluten-sensitive enteropathy. (10548367)
1999
17
Dermatitis herpetiformis intolerant to dapsone in Aids. (20885054)
1999
18
The dapsone hypersensitivity syndrome occurring in a patient with dermatitis herpetiformis. (9390358)
1997
19
Palmar purpura: an atypical presentation of childhood dermatitis herpetiformis. (7899181)
1994
20
Confocal laser scanning microscopic and immunoelectron microscopic studies of the anatomical distribution of fibrillar IgA deposits in dermatitis herpetiformis. (8466215)
1993
21
Dermatitis herpetiformis and primary sclerosing cholangitis. (8403478)
1993
22
HLA-DQ2 second-domain polymorphisms may explain increased trans-associated risk in celiac disease and dermatitis herpetiformis. (8138424)
1993
23
Acquired cutis laxa with dermatitis herpetiformis and sarcoidosis. (8408824)
1993
24
Immunoglobulin and HLA-DP genes contribute to the susceptibility to juvenile dermatitis herpetiformis. (1627534)
1992
25
Coeliac-type dental enamel defects in patients with dermatitis herpetiformis. (1350136)
1992
26
Asymptomatic anemia in three men with dermatitis herpetiformis. (1560069)
1992
27
Helicobacter pylori serology in patients with coeliac disease and dermatitis herpetiformis. (1517459)
1992
28
Role of elemental diet in dermatitis herpetiformis. (1935210)
1991
29
Immunogenetics of dermatitis herpetiformis. (1806221)
1991
30
Gluten-free diet in dermatitis herpetiformis. (1991004)
1991
31
Sulfasalazine in dermatitis herpetiformis. (1969428)
1990
32
Similarities in intestinal humoral immunity in dermatitis herpetiformis without enteropathy and in coeliac disease. (1972433)
1990
33
Perimenstrual nonvesicular dermatitis herpetiformis. (2303587)
1990
34
Normal IgA production by peripheral blood lymphocytes in dermatitis herpetiformis and linear IgA dermatosis. (2919896)
1989
35
Vitronectin colocalizes with Ig deposits and C9 neoantigen in discoid lupus erythematosus and dermatitis herpetiformis, but not in bullous pemphigoid. (2474318)
1989
36
Isotype distribution and serial levels of antibodies reactive with dietary protein antigens in dermatitis herpetiformis. (2641534)
1989
37
Paediatric coeliac disease and dermatitis herpetiformis: differences in complement activation in relation to diet. (2491289)
1989
38
Intestinal permeability in dermatitis herpetiformis. (3397588)
1988
39
Cellobiose/mannitol sugar absorption test in patients with dermatitis herpetiformis: a preliminary report. (3138803)
1988
40
Effects of additional dietary gluten on the small-intestinal mucosa of volunteers and of patients with dermatitis herpetiformis. (3629179)
1987
41
The cause of dermatitis herpetiformis. (3813599)
1987
42
Primary biliary cirrhosis in a diabetic male with dermatitis herpetiformis. (3652504)
1987
43
Immunoblotting analysis of antigliadin antibodies in the sera of patients with dermatitis herpetiformis and gluten-sensitive enteropathy. (3710610)
1986
44
Recurrent "flare" of dermatitis herpetiformis after cytotoxic therapy for malignant lymphoma. (3528778)
1986
45
The immunopathogenesis of dermatitis herpetiformis. (3981033)
1985
46
Immunoelectron microscopic findings in oral mucosa of patients with dermatitis herpetiformis and linear IgA disease. (3895376)
1985
47
Juvenile dermatitis herpetiformis. (375840)
1979
48
Adult coeliac disease. A case with ulceration, dermatitis herpetiformis and reticulosarcoma. (766153)
1976
49
Juvenile dermatitis herpetiformis versus "benign chronic bullous dermatosis of childhood." Are these immunologic diseases? (1104719)
1975
50
Juvenile dermatitis herpetiformis in Jamaica: clinical and gastrointestinal features. (5018677)
1972

Genetic Variations for Dermatitis Herpetiformis

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Expression for genes affiliated with Dermatitis Herpetiformis

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dermatitis Herpetiformis

Search GEO for disease gene expression data for Dermatitis Herpetiformis.

Pathways for genes affiliated with Dermatitis Herpetiformis

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Sources:
37NCBI BioSystems Database, 51QIAGEN, 53Reactome, 12EMD Millipore
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Compounds for genes affiliated with Dermatitis Herpetiformis

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

(show top 50)    (show all 56)
idCompoundScoreTop Affiliating Genes
1glutamate4410.8FBN1
2gliadin4410.7CD79A, TGM2
3pge24410.7PLAU, CCL11
4indomethacin44 59 28 1113.6EDNRB, PLAU
5tgm 24410.6TGM2, TGM3
6n-isobutyl-n-[4-methoxyphenylsulfonyl]glycyl hydroxamic acid44 1111.6MMP12, MMP3
7gm 60015910.5MMP7, MMP12, MMP3
8uk 3833675910.5MMP3, MMP12, MMP7
9gi 254023x5910.5MMP12, MMP7, MMP3
10unicap4410.5CCL11, CD79A
11etanercept44 49 1112.5IL2RB, MMP3, MMP12
12marimastat44 59 1112.5MMP3, MMP7, PLAU, MMP12
13batimastat44 59 1112.5MMP3, PLAU, MMP7, MMP12
14prednisolone44 28 1112.4CCL11, MMP3, CD79A, IL2RB
15methacholine44 1111.4CD79A, CCL11, IL2RB
16azathioprine44 49 2 1113.4CD79A, EDNRB, IL2RB, PLAU
17l-glutamine28 11 2412.4TGM1, TGM2, TGM6, TGM3
18sodium dodecylsulfate4410.4CD79A, PLAU, TGM1, FBN1
19hyaluronic acid44 2411.4FBN1, CD79A, MMP3, PLAU, CCL11
20epsilon-(gamma-glutamyl)lysine4410.4TGM1, TGM2
21dexamethasone44 49 28 1113.3CCL11, CD79A, EDNRB, MMP12
22matrigel4410.3MMP7, PLAU, MMP12, MMP3, CCL11
23phorbol4410.3TRB, PLAU, TGM1, MMP3
24histamine44 28 2412.3IL2RB, CD79A, CCL11, PLAU, MMP3
25n acetylcysteine4410.3CCL11, CD79A, IL2RB, PLAU, MMP12, MMP3
26fibrinogen4410.3MMP12, CD79A, MMP7, PLAU, TGM2
27cyclosporin a44 28 5912.3CCL11, EDNRB, TRB, IL2RB, CD79A
28gluten4410.3TGM2, CD79A
29genistein44 28 59 2 11 2415.3CCL11, MMP3, IL2RB, MMP12, PLAU
30dmso4410.2MMP7, MMP3, PLAU, EDNRB
31oxygen44 2411.2CCL11, TGM2, MMP12, EDNRB, FBN1, IL2RB
32doxycycline44 1111.2MMP3, PLAU, CD79A
33aspirin44 49 28 2413.2PLAU, IL2RB, CD79A, CCL11
34glutamine4410.2MMP3, TGM1, TGM2, PLAU, HLA-DQB1
35serine4410.2EDNRB, MMP12, FBN1, IL2RB, TPSB2, MMP3
36paraffin4410.2TRB, MMP3, PLAU, CD79A, MMP7
37steroid4410.1TGM1, PLAU, CCL11, CD79A, EDNRB, IL2RB
38progesterone44 59 28 11 2414.1IL2RB, MMP3, MMP7, PLAU, TGM1, FBN1
39heparin44 28 11 2413.1MMP3, CD79A, CCL11, FBN1
40arginine4410.0EDNRB, FBN1, CD79A, IL2RB, PLAU, TGM1
41alanine4410.0TGM2, MMP3, IL2RB, CD79A, FBN1, EDNRB
42vegf4410.0IL2RB, CCL11, MMP12, PLAU, MMP7, EDNRB
43phosphatidylinositol4410.0CCL11, PLAU, MMP12, IL2RB, EDNRB, MMP7
44cysteine4410.0TGM2, PLAU, MMP7, MMP3, CD79A, FBN1
45nitric oxide44 11 2411.9IL2RB, MMP7, CCL11, TGM1, MMP12, EDNRB
46retinoid449.9TGM2, IL2RB, PLAU, TGM1
47retinoic acid44 2410.8TGM1, PLAU, MMP7, MMP3, IL2RB, CD79A
48ribonucleic acid449.7MMP3, PLAU, IL2RB, MMP7
49tyrosine449.7FBN1, MMP7, MMP12, IL2RB, TRB, MMP3
50calcium44 49 11 2412.4IL2RB, TRB, FBN1, EDNRB, CCL11, TGM3

GO Terms for genes affiliated with Dermatitis Herpetiformis

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16Gene Ontology
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Cellular components related to Dermatitis Herpetiformis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1proteinaceous extracellular matrixGO:00557810.0MMP7, MMP12, MMP3, FBN1
2extracellular regionGO:0055769.1TPSB2, CCL11, FBN1, CGREF1, IGHA1, MMP3

Biological processes related to Dermatitis Herpetiformis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell envelope organizationGO:04316310.3TGM3, TGM1
2peptide cross-linkingGO:01814910.2TGM3, TGM6, TGM1
3collagen catabolic processGO:03057410.0MMP3, MMP12, MMP7
4extracellular matrix organizationGO:03019810.0MMP7, MMP12, MMP3, FBN1
5proteolysisGO:0065089.9MMP3, MMP12, MMP7, PLAU, TPSB2
6extracellular matrix disassemblyGO:0226179.8MMP7, MMP12, MMP3

Molecular functions related to Dermatitis Herpetiformis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein-glutamine gamma-glutamyltransferase activityGO:0038109.9TGM3, TGM6, TGM2, TGM1
2calcium ion bindingGO:0055099.8TGM3, MMP12, MMP3, CGREF1, FBN1
3metalloendopeptidase activityGO:0042229.8MMP3, MMP12, MMP7

Products for genes affiliated with Dermatitis Herpetiformis

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Dermatitis Herpetiformis

About this section
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