MCID: DRM007
MIFTS: 64

Dermatitis Herpetiformis malady

Rare diseases, Skin diseases categories

Summaries for Dermatitis Herpetiformis

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NIH Rare Diseases:43 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 durhing-brocq 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:65 Dermatitis herpetiformis (DH), or Duhring\'s disease, is a chronic blistering skin condition,... more...

Aliases & Classifications for Dermatitis Herpetiformis

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Sources:
10Disease Ontology, 43NIH Rare Diseases, 12DISEASES, 45Novoseek, 49Orphanet, 62UMLS, 29ICD9CM, 57SNOMED-CT, 35MeSH, 40NCIt, 36MESH via Orphanet, 28ICD10 via Orphanet, 63UMLS via Orphanet, 27ICD10
See all sources

Dermatitis Herpetiformis, Aliases & Descriptions:

Name: Dermatitis Herpetiformis 10 43 12 45 49 62
Durhing-Brocq Disease 43 49
Duhring's Disease 10 43
 
Dermatosis Herpetiformis 10
Duhring Brocq Disease 43
Brocq-Duhring Disease 43


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 49 
Rare skin diseases


Characteristics (Orphanet epidemiological data):

49
dermatitis herpetiformis:
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe),1-9/1000000 (Italy),1-9/100000 (Ireland),1-5/10000 (Ireland); Age of onset: All ages


External Ids:

Disease Ontology10 DOID:8505
ICD9CM29 694.0
MeSH35 D003874
NCIt40 C26742
Orphanet49 1656
MESH via Orphanet36 D003874
ICD10 via Orphanet28 L13.0
UMLS via Orphanet63 C0011608
ICD1027 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 146)
idRelated DiseaseScoreTop Affiliating Genes
1dermatitis32.3CCL11, CD79A
2celiac disease31.4CD79A, MMP12, TGM2
3bullous pemphigoid31.2CCL11, PLAU, CD79A
4ulcerative colitis30.7MMP3, CCL11, CD79A
5arthritis30.5MMP12, MMP3, IL2RB
6pemphigus30.5CD79A, PLAU
7primary biliary cirrhosis30.4CD79A, IL2RB
8systemic lupus erythematosus30.4CD79A, IL2RB, MMP3
9rheumatoid arthritis30.3MMP12, MMP3, CCL11, IL2RB, CD79A
10epidermolysis bullosa acquisita30.3CD79A
11gastroenteritis30.3CD79A
12lung cancer30.1PLAU, MMP12, TGM2
13juvenile dermatitis herpetiformis10.8
14linear iga disease10.6
15lupus erythematosus10.5
16colitis10.5
17thyroiditis10.5
18herpes gestationis10.4
19gastritis10.4
20psoriasis10.4
21discoid lupus erythematosus10.4
22duodenitis10.4
23sclerosing cholangitis10.4
24cholangitis10.4
25epidermolysis bullosa10.4
26anaplastic large cell lymphoma10.3
27glomerulonephritis10.3
28atrophic gastritis10.3
29protein-losing enteropathy10.3
30subcorneal pustular dermatosis10.3
31impetigo herpetiformis10.3
32stiff-person syndrome10.3
33cutaneous t cell lymphoma10.3
34impetigo10.3
35intestinal disease10.3
36laryngitis10.3
37pancreatitis10.3
38purpura10.3
39urticaria10.3
40erythema elevatum diutinum10.3
41ataxia10.3
42endotheliitis10.3
43psoriatic arthritis10.1
44acne10.1
45chronic myelomonocytic leukemia10.1
46hepatitis10.1
47leukemia10.1
48myasthenia gravis10.1
49neuromyelitis optica10.1
50pancreatic somatostatinoma10.1

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:

 49 (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 12)
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

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 UMLS62/NDF-RT41:

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:

33
Skin, Bone, T cells, Thyroid, Small intestine, 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:

37
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 558)
idTitleAuthorsYear
1
Regulatory T cells as well as IL-10 are reduced in the skin of patients with dermatitis herpetiformis. (25465638)
2015
2
Evidence for a role of autoantibodies to heat shock protein 60, 70, and 90 in patients with dermatitis herpetiformis. (24643797)
2014
3
Transverse myelitis associated with an itchy rash and hyperckemia: neuromyelitis optica associated with dermatitis herpetiformis. (24637913)
2014
4
Dermatitis herpetiformis in Brazilan male celiac disease patients: A case series. (25544421)
2014
5
IL-17 expression in dermatitis herpetiformis and bullous pemphigoid. (23970818)
2013
6
Does dermatitis herpetiformis result in bone loss as coeliac disease does? A cross sectional study. (23859446)
2013
7
Management of dermatitis herpetiformis. (22560140)
2012
8
Long-term combination therapy of ustekinumab and dapsone in a patient with psoriasis and dermatitis herpetiformis Duhring. (22784106)
2012
9
Autoimmunity-driven enzymatic remodeling of the dermal-epidermal junction in bullous pemphigoid and dermatitis herpetiformis. (21916544)
2012
10
The long-term risk of malignancy following a diagnosis of coeliac disease or dermatitis herpetiformis: a cohort study. (22288441)
2012
11
Reduced mortality in dermatitis herpetiformis: a population-based study of 476 patients. (22708883)
2012
12
Does Adam17 cause the destruction of anchoring fibers via shedding tumor necrosis factor I+ in bullous pemphigoid and dermatitis herpetiformis? (22713436)
2012
13
Discontinuing dapsone treatment and reintroducing dietary gluten in patients with dermatitis herpetiformis in remission: comment on "remission in dermatitis herpetiformis". (21422338)
2011
14
The falling incidence and prevalence of dermatitis herpetiformis. (21777210)
2011
15
Inflammatory epidermolysis bullosa acquisita mimicking toxic epidermal necrolysis and dermatitis herpetiformis. (19663858)
2009
16
No increase in risk of fracture, malignancy or mortality in dermatitis herpetiformis: a cohort study. (18315580)
2008
17
Might dermatitis herpetiformis be associated with discoid lupus erythematosus? (18482033)
2008
18
Childhood dermatitis herpetiformis: a case report and review of the literature. (18246879)
2007
19
Permeability, zonulin production, and enteropathy in dermatitis herpetiformis. (15822038)
2005
20
A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice. (15489956)
2004
21
Parallel expression of macrophage metalloelastase (MMP-12) in duodenal and skin lesions of patients with dermatitis herpetiformis. (11247893)
2001
22
Concordance of dermatitis herpetiformis and celiac disease in monozygous twins. (11121131)
2000
23
Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide. (10844495)
2000
24
Tissue transglutaminase antibodies in dermatitis herpetiformis. (10490368)
1999
25
Hemidesmosomal molecular changes in dermatitis herpetiformis; decreased expression of BP230 and plectin/HD1 in uninvolved skin. (10416682)
1999
26
Th2-like cytokine activity in dermatitis herpetiformis. (9602868)
1998
27
Risk factors for ischaemic heart disease in patients with dermatitis herpetiformis. (9204017)
1997
28
Dermatitis herpetiformis following gastric surgery. (9006392)
1997
29
Dermatitis herpetiformis induced by nonsteroidal anti-inflammatory drugs. (8169023)
1994
30
IgA antibodies against reticulin and endomysium in the serum and gastrointestinal secretions of patients with dermatitis herpetiformis. (8049567)
1994
31
Efficacy of cyclosporine in two patients with dermatitis herpetiformis resistant to conventional therapy. (8496445)
1993
32
Increased jejunal intraepithelial lymphocytes bearing gamma/delta T-cell receptor in dermatitis herpetiformis. (1314748)
1992
33
Role of elemental diet in dermatitis herpetiformis. (1935210)
1991
34
Intestinal humoral immunity in dermatitis herpetiformis. (1975360)
1990
35
Laryngeal involvement in dermatitis herpetiformis. (2312825)
1990
36
Manic depression induced by dapsone in patient with dermatitis herpetiformis. (2510873)
1989
37
Normal IgA production by peripheral blood lymphocytes in dermatitis herpetiformis and linear IgA dermatosis. (2919896)
1989
38
Dermatitis herpetiformis. (3153433)
1988
39
Dermatitis herpetiformis: a case with palmar purpuric lesions treated with disodium cromoglycate. (3139724)
1988
40
Aggressive gluten challenge of dermatitis herpetiformis cases converts them from seronegative to seropositive for IgA-class endomysial antibodies. (3372760)
1988
41
Dermatitis herpetiformis and hemodialysis. (3678553)
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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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
1gluten4510.0CD79A, TGM2
2unicap4510.0CCL11, CD79A
3reticulin459.9TGM2, CD79A
4sodium cromoglycate459.9CD79A, CCL11
5gliadin459.9TGM2, CD79A
6n-isobutyl-n-[4-methoxyphenylsulfonyl]glycyl hydroxamic acid45 1310.6MMP3, MMP12
7clarithromycin45 1310.6CCL11, CD79A
8piroxicam45 1310.5CD79A, MMP3
9nimesulide45 1310.5PLAU, MMP3
10uk 383367619.4MMP12, MMP3
11methacholine45 51 1311.4CD79A, IL2RB, CCL11
12gi 254023x619.4MMP12, MMP3
13azathioprine45 3 51 1312.3CD79A, PLAU, IL2RB
14gm 6001619.3MMP12, MMP3
15mnng459.3PLAU, MMP3
16tacrolimus45 51 1311.3CCL11, IL2RB, CD79A
17doxycycline45 1310.2MMP3, PLAU, CD79A
18marimastat45 61 1311.2PLAU, MMP3, MMP12
19batimastat45 61 1311.1MMP12, MMP3, PLAU
20fibrinogen459.1TGM2, MMP12, PLAU, CD79A
21retinoid459.1PLAU, IL2RB, TGM2
22etanercept45 51 1311.0MMP12, MMP3, IL2RB
23adalimumab45 51 1311.0CD79A, MMP3
24methotrexate51 45 1310.9CD79A, IL2RB, MMP3
25aspirin45 51 30 2611.9CCL11, IL2RB, PLAU, CD79A
26steroid458.8CCL11, IL2RB, PLAU, CD79A
27rantes458.7MMP3, CCL11, IL2RB
28phosphatidylinositol458.7PLAU, IL2RB, CCL11, MMP12
29hyaluronic acid45 269.7MMP3, CCL11, PLAU, CD79A
30indomethacin45 30 61 1311.7CD79A, PLAU, CCL11, MMP3
31matrigel458.6PLAU, CCL11, MMP3, MMP12
32heparin45 30 26 1311.6MMP3, CCL11, PLAU, CD79A
33prednisolone45 30 1310.6CD79A, IL2RB, CCL11, MMP3
34glutamate458.6TGM2, MMP3, PLAU, CD79A
35cyclosporin a45 30 6110.5MMP3, CCL11, IL2RB, CD79A
36lactate458.5CD79A, PLAU, IL2RB, MMP3
3712-o-tetradecanoylphorbol 13-acetate458.5PLAU, IL2RB, MMP3
38creatinine458.5MMP3, IL2RB, PLAU, CD79A
39ribonucleic acid458.4MMP3, IL2RB, PLAU
40dexamethasone45 51 30 1311.2MMP12, MMP3, CCL11, IL2RB, CD79A
41histamine45 30 2610.1MMP3, CCL11, IL2RB, PLAU, CD79A
42pge2458.1MMP3, CCL11, IL2RB, PLAU, CD79A
43oxygen45 269.0TGM2, MMP12, MMP3, CCL11, IL2RB
44genistein45 30 61 3 26 1313.0MMP12, MMP3, CCL11, IL2RB, PLAU
45serine458.0TGM2, MMP12, MMP3, IL2RB, PLAU
46tyrosine457.9MMP12, MMP3, CCL11, IL2RB, PLAU
47n acetylcysteine457.7CD79A, PLAU, IL2RB, CCL11, MMP3, MMP12
48vegf457.7MMP12, MMP3, CCL11, IL2RB, PLAU, CD79A
49alanine457.6TGM2, MMP3, CCL11, IL2RB, PLAU, CD79A
50retinoic acid45 268.6TGM2, MMP3, CCL11, IL2RB, PLAU, CD79A

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:00055768.2PLAU, CCL11, MMP3, MMP12

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

idNameGO IDScoreTop Affiliating Genes
1extracellular matrix disassemblyGO:00226179.2MMP3, MMP12
2collagen catabolic processGO:00305748.9MMP3, MMP12
3proteolysisGO:00065088.6PLAU, MMP3, MMP12

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

idNameGO IDScoreTop Affiliating Genes
1metalloendopeptidase activityGO:00042229.2MMP3, MMP12

Products for genes affiliated with Dermatitis Herpetiformis

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Sources for Dermatitis Herpetiformis

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4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet