MCID: DRM007
MIFTS: 65

Dermatitis Herpetiformis malady

Rare diseases, Skin diseases, Immune diseases categories

Summaries for Dermatitis Herpetiformis

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NIH Rare Diseases:42 Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. these are more common on the knees, elbows, buttocks and shoulder blades. the slow onset of symptoms usually begins during adulthood, but children can also be affected. other symptoms may include fluid-filled sores; red lesions that resemble hives; and itchiness, redness and burning. the exact cause of this disease is not known, but it is frequently associated with the inability to digest gluten. people with this disease are typically treated with the drug dapsone. last updated: 10/29/2014

MalaCards based summary: Dermatitis Herpetiformis, also known as duhring's disease, is related to dermatitis and celiac disease, and has symptoms including pruritus, urticaria and hypermelanotic macule. An important gene associated with Dermatitis Herpetiformis is CD79A (CD79a molecule, immunoglobulin-associated alpha), and among its related pathways are Matrix Metalloproteinases and GPCR Pathway. The drugs dapsone and sulfapyridine pwdr and the compounds gluten and unicap have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and t cells, and related mouse phenotypes are homeostasis/metabolism and nervous system.

Wikipedia:64 Dermatitis herpetiformis (DH), or Duhring\'s disease, is a chronic blistering skin condition,... more...

Description from OMIM:46 601230

Aliases & Classifications for Dermatitis Herpetiformis

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Sources:
9Disease Ontology, 42NIH Rare Diseases, 11DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 61UMLS, 56SNOMED-CT, 28ICD9CM, 34MeSH, 39NCIt, 35MESH via Orphanet, 27ICD10 via Orphanet, 62UMLS via Orphanet, 26ICD10
See all sources

Dermatitis Herpetiformis, Aliases & Descriptions:

Name: Dermatitis Herpetiformis 9 42 11 44 48 46 61
Duhring's Disease 9 42
Dermatosis Herpetiformis 9
 
Duhring Brocq Disease 42
Brocq-Duhring Disease 42
Durhing-Brocq Disease 48


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases, Immune diseases
Orphanet: 48 
Rare skin diseases


Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology9 DOID:8505
ICD9CM28 694.0
MeSH34 D003874
NCIt39 C26742
OMIM46 601230
MESH via Orphanet35 D003874
ICD10 via Orphanet27 L13.0
UMLS via Orphanet62 C0011608
ICD1026 L13.0

Related Diseases for Dermatitis Herpetiformis

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Diseases in the Dermatitis Herpetiformis family:

Juvenile Dermatitis Herpetiformis

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

(show top 50)    (show all 153)
idRelated DiseaseScoreTop Affiliating Genes
1dermatitis32.5CCL11, CD79A
2celiac disease31.5TGM2, MMP12, CD79A
3bullous pemphigoid31.2CD79A, PLAU, CCL11
4epidermolysis bullosa acquisita30.9CD79A
5gastroenteritis30.9CD79A
6pemphigus30.8CD79A, PLAU
7ulcerative colitis30.6CD79A, CCL11, MMP3
8primary biliary cirrhosis30.4IL2RB, CD79A
9ankylosing spondylitis30.4CD79A, MMP3
10arthritis30.3IL2RB, MMP3, MMP12
11sarcoidosis30.2IL2RB, CCL11
12systemic lupus erythematosus30.2CD79A, MMP3, IL2RB
13lung cancer29.8TGM2, MMP12, PLAU
14rheumatoid arthritis29.6CCL11, MMP3, IL2RB, MMP12, CD79A
15juvenile dermatitis herpetiformis10.7
16linear iga disease10.6
17lupus erythematosus10.5
18poems syndrome10.5CD79A
19conjunctivitis10.5CD79A
20exanthem10.5CD79A
21cryptosporidiosis10.5CD79A
22bronchitis10.5CD79A
23vitiligo10.5
24colitis10.5
25thyroiditis10.5
26corneal ulcer10.4CCL11
27herpes gestationis10.4
28gastritis10.4
29psoriasis10.4
30discoid lupus erythematosus10.4
31uveitis10.4CCL11
32sclerosing cholangitis10.4
33duodenitis10.4
34cholangitis10.4
35epidermolysis bullosa10.4
36urticaria10.4
37primary sclerosing cholangitis10.4
38glucose intolerance10.3CD79A, TGM2
39anaplastic large cell lymphoma10.3
40diabetes mellitus10.3
41glomerulonephritis10.3
42stiff-person syndrome10.3
43atrophic gastritis10.3
44protein-losing enteropathy10.3
45subcorneal pustular dermatosis10.3
46impetigo herpetiformis10.3
47cutaneous t cell lymphoma10.3
48impetigo10.3
49intestinal disease10.3
50laryngitis10.3

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



Diseases related to dermatitis herpetiformis

Symptoms for Dermatitis Herpetiformis

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Symptoms by clinical synopsis from OMIM:

601230

Clinical features from OMIM:

601230

Symptoms:

 48 (show all 15)
  • urticaria
  • 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
  • cutaneous edema
  • bone pain

HPO human phenotypes related to Dermatitis Herpetiformis:

(show all 15)
id Description Frequency HPO Source Accession
1 pruritus hallmark (90%) HP:0000989
2 urticaria hallmark (90%) HP:0001025
3 hypermelanotic macule hallmark (90%) HP:0001034
4 microcytic anemia hallmark (90%) HP:0001935
5 malabsorption hallmark (90%) HP:0002024
6 recurrent fractures hallmark (90%) HP:0002757
7 autoimmunity hallmark (90%) HP:0002960
8 abnormal blistering of the skin hallmark (90%) HP:0008066
9 eczema typical (50%) HP:0000964
10 edema occasional (7.5%) HP:0000969
11 bone pain occasional (7.5%) HP:0002653
12 lichenification occasional (7.5%) HP:0100725
13 autosomal dominant inheritance HP:0000006
14 eczema HP:0000964
15 abnormality of metabolism/homeostasis HP:0001939

Drugs & Therapeutics for Dermatitis Herpetiformis

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Drug clinical trials:

Search ClinicalTrials for Dermatitis Herpetiformis

Search NIH Clinical Center for Dermatitis Herpetiformis

Inferred drug relations via UMLS61/NDF-RT40:

Genetic Tests for Dermatitis Herpetiformis

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Anatomical Context for Dermatitis Herpetiformis

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MalaCards organs/tissues related to Dermatitis Herpetiformis:

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

Animal Models for Dermatitis Herpetiformis or affiliated genes

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MGI Mouse Phenotypes related to Dermatitis Herpetiformis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053767.9CD79A, PLAU, MMP3, MMP12, TGM2
2MP:00036317.6TGM2, MMP12, MMP3, PLAU, CD79A
3MP:00053976.7CD79A, TGM2, MMP12, MMP3, CCL11, IL2RB
4MP:00053876.6TGM2, MMP12, MMP3, CCL11, IL2RB, PLAU

Publications for Dermatitis Herpetiformis

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Articles related to Dermatitis Herpetiformis:

(show top 50)    (show all 549)
idTitleAuthorsYear
1
Evidence for a role of autoantibodies to heat shock protein 60, 70, and 90 in patients with dermatitis herpetiformis. (24643797)
2014
2
Transverse myelitis associated with an itchy rash and hyperckemia: neuromyelitis optica associated with dermatitis herpetiformis. (24637913)
2014
3
IL-17 expression in dermatitis herpetiformis and bullous pemphigoid. (23970818)
2013
4
Does dermatitis herpetiformis result in bone loss as coeliac disease does? A cross sectional study. (23859446)
2013
5
Management of dermatitis herpetiformis. (22560140)
2012
6
Long-term combination therapy of ustekinumab and dapsone in a patient with psoriasis and dermatitis herpetiformis Duhring. (22784106)
2012
7
Autoimmunity-driven enzymatic remodeling of the dermal-epidermal junction in bullous pemphigoid and dermatitis herpetiformis. (21916544)
2012
8
The long-term risk of malignancy following a diagnosis of coeliac disease or dermatitis herpetiformis: a cohort study. (22288441)
2012
9
Reduced mortality in dermatitis herpetiformis: a population-based study of 476 patients. (22708883)
2012
10
Does Adam17 cause the destruction of anchoring fibers via shedding tumor necrosis factor I+ in bullous pemphigoid and dermatitis herpetiformis? (22713436)
2012
11
Discontinuing dapsone treatment and reintroducing dietary gluten in patients with dermatitis herpetiformis in remission: comment on "remission in dermatitis herpetiformis". (21422338)
2011
12
Acupuncture and a gluten-free diet relieve urticaria and eczema in a case of undiagnosed dermatitis herpetiformis and atypical or extraintestinal celiac disease: a case report. (22654688)
2011
13
The falling incidence and prevalence of dermatitis herpetiformis. (21777210)
2011
14
Inflammatory epidermolysis bullosa acquisita mimicking toxic epidermal necrolysis and dermatitis herpetiformis. (19663858)
2009
15
No increase in risk of fracture, malignancy or mortality in dermatitis herpetiformis: a cohort study. (18315580)
2008
16
Might dermatitis herpetiformis be associated with discoid lupus erythematosus? (18482033)
2008
17
Childhood dermatitis herpetiformis: a case report and review of the literature. (18246879)
2007
18
Permeability, zonulin production, and enteropathy in dermatitis herpetiformis. (15822038)
2005
19
A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice. (15489956)
2004
20
Parallel expression of macrophage metalloelastase (MMP-12) in duodenal and skin lesions of patients with dermatitis herpetiformis. (11247893)
2001
21
Concordance of dermatitis herpetiformis and celiac disease in monozygous twins. (11121131)
2000
22
Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide. (10844495)
2000
23
Tissue transglutaminase antibodies in dermatitis herpetiformis. (10490368)
1999
24
Hemidesmosomal molecular changes in dermatitis herpetiformis; decreased expression of BP230 and plectin/HD1 in uninvolved skin. (10416682)
1999
25
Th2-like cytokine activity in dermatitis herpetiformis. (9602868)
1998
26
Risk factors for ischaemic heart disease in patients with dermatitis herpetiformis. (9204017)
1997
27
Dermatitis herpetiformis following gastric surgery. (9006392)
1997
28
Dermatitis herpetiformis induced by nonsteroidal anti-inflammatory drugs. (8169023)
1994
29
IgA antibodies against reticulin and endomysium in the serum and gastrointestinal secretions of patients with dermatitis herpetiformis. (8049567)
1994
30
Efficacy of cyclosporine in two patients with dermatitis herpetiformis resistant to conventional therapy. (8496445)
1993
31
Increased jejunal intraepithelial lymphocytes bearing gamma/delta T-cell receptor in dermatitis herpetiformis. (1314748)
1992
32
Role of elemental diet in dermatitis herpetiformis. (1935210)
1991
33
Intestinal humoral immunity in dermatitis herpetiformis. (1975360)
1990
34
Laryngeal involvement in dermatitis herpetiformis. (2312825)
1990
35
Manic depression induced by dapsone in patient with dermatitis herpetiformis. (2510873)
1989
36
Normal IgA production by peripheral blood lymphocytes in dermatitis herpetiformis and linear IgA dermatosis. (2919896)
1989
37
Dermatitis herpetiformis. (3153433)
1988
38
Dermatitis herpetiformis: a case with palmar purpuric lesions treated with disodium cromoglycate. (3139724)
1988
39
Aggressive gluten challenge of dermatitis herpetiformis cases converts them from seronegative to seropositive for IgA-class endomysial antibodies. (3372760)
1988
40
Dermatitis herpetiformis and hemodialysis. (3678553)
1987
41
Dermatitis herpetiformis: relation between circulating immune complexes, small-intestinal mucosal status, and immunohistopathological findings. (3632036)
1987
42
IgA endomysium antibody in children with dermatitis herpetiformis treated with gluten-free diet. (3774647)
1986
43
Anti-endomysial antibodies. A serologic marker of dermatitis herpetiformis. (3513713)
1986
44
The relationship between dapsone dose, serum concentration and disease severity in dermatitis herpetiformis. (3954818)
1986
45
Characterization of circulating and cutaneous IgA immune complexes in patients with dermatitis herpetiformis. (4020133)
1985
46
Thyroid abnormalities in dermatitis herpetiformis. Prevalence of clinical thyroid disease and thyroid autoantibodies. (3966757)
1985
47
Intestinal permeability in patients with coeliac disease and dermatitis herpetiformis. (3934051)
1985
48
Atypical dermatitis herpetiformis in two patients with internal malignancy. (6744651)
1984
49
Adult coeliac disease. A case with ulceration, dermatitis herpetiformis and reticulosarcoma. (766153)
1976
50
Juvenile dermatitis herpetiformis (a case report). (4777767)
1973

Variations for Dermatitis Herpetiformis

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Expression for genes affiliated with Dermatitis Herpetiformis

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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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Compounds for genes affiliated with Dermatitis Herpetiformis

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Compounds related to Dermatitis Herpetiformis according to GeneCards/GeneDecks:

(show top 50)    (show all 84)
idCompoundScoreTop Affiliating Genes
1gluten4410.0CD79A, TGM2
2unicap4410.0CD79A, CCL11
3reticulin449.9CD79A, TGM2
4sodium cromoglycate449.9CCL11, CD79A
5gliadin449.9CD79A, TGM2
6n-isobutyl-n-[4-methoxyphenylsulfonyl]glycyl hydroxamic acid44 1210.6MMP12, MMP3
7clarithromycin44 1210.6CCL11, CD79A
8piroxicam44 1210.5CD79A, MMP3
9nimesulide44 1210.5PLAU, MMP3
10uk 383367609.4MMP12, MMP3
11methacholine44 50 1211.4IL2RB, CD79A, CCL11
12gi 254023x609.4MMP12, MMP3
13azathioprine44 3 50 1212.3CD79A, IL2RB, PLAU
14gm 6001609.3MMP12, MMP3
15mnng449.3PLAU, MMP3
16tacrolimus44 50 1211.3CCL11, IL2RB, CD79A
17doxycycline44 1210.2MMP3, PLAU, CD79A
18marimastat44 60 1211.2MMP12, PLAU, MMP3
19batimastat44 60 1211.1PLAU, MMP3, MMP12
20fibrinogen449.1PLAU, CD79A, TGM2, MMP12
21retinoid449.1PLAU, IL2RB, TGM2
22etanercept44 50 1211.0MMP12, IL2RB, MMP3
23adalimumab44 50 1211.0CD79A, MMP3
24methotrexate50 44 1210.9MMP3, IL2RB, CD79A
25aspirin44 50 29 2511.9CCL11, CD79A, IL2RB, PLAU
26steroid448.8PLAU, CCL11, IL2RB, CD79A
27rantes448.7CCL11, IL2RB, MMP3
28phosphatidylinositol448.7MMP12, IL2RB, PLAU, CCL11
29hyaluronic acid44 259.7CD79A, PLAU, MMP3, CCL11
30indomethacin44 29 60 1211.7MMP3, CCL11, CD79A, PLAU
31matrigel448.6PLAU, MMP12, MMP3, CCL11
32heparin44 29 25 1211.6CD79A, PLAU, MMP3, CCL11
33prednisolone44 29 1210.6MMP3, IL2RB, CD79A, CCL11
34glutamate448.6PLAU, CD79A, TGM2, MMP3
35cyclosporin a44 29 6010.5MMP3, CD79A, CCL11, IL2RB
36lactate448.5MMP3, IL2RB, CD79A, PLAU
3712-o-tetradecanoylphorbol 13-acetate448.5IL2RB, PLAU, MMP3
38creatinine448.5IL2RB, PLAU, CD79A, MMP3
39ribonucleic acid448.4PLAU, MMP3, IL2RB
40dexamethasone44 50 29 1211.2CD79A, MMP12, IL2RB, CCL11, MMP3
41histamine44 29 2510.1PLAU, IL2RB, CCL11, MMP3, CD79A
42pge2448.1PLAU, IL2RB, MMP3, CD79A, CCL11
43oxygen44 259.0CCL11, IL2RB, MMP3, MMP12, TGM2
44genistein44 29 60 3 25 1213.0CCL11, MMP3, MMP12, IL2RB, PLAU
45serine448.0MMP3, IL2RB, TGM2, MMP12, PLAU
46tyrosine447.9MMP3, CCL11, IL2RB, PLAU, MMP12
47n acetylcysteine447.7MMP3, IL2RB, CD79A, PLAU, CCL11, MMP12
48vegf447.7MMP3, PLAU, MMP12, CD79A, IL2RB, CCL11
49alanine447.6MMP3, CCL11, TGM2, IL2RB, PLAU, CD79A
50retinoic acid44 258.6IL2RB, CD79A, PLAU, CCL11, MMP3, TGM2

GO Terms for genes affiliated with Dermatitis Herpetiformis

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Cellular components related to Dermatitis Herpetiformis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.2PLAU, CCL11, MMP3, MMP12

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

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix disassemblyGO:0226179.2MMP3, MMP12
2collagen catabolic processGO:0305748.9MMP3, MMP12
3proteolysisGO:0065088.6PLAU, MMP3, MMP12

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

idNameGO IDScoreTop Affiliating Genes
1metalloendopeptidase activityGO:0042229.2MMP3, MMP12

Products for genes affiliated with Dermatitis Herpetiformis

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Sources for Dermatitis Herpetiformis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
29IUPHAR
30KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet