DH
MCID: DRM007
MIFTS: 54

Dermatitis Herpetiformis (DH)

Categories: Genetic diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Dermatitis Herpetiformis

MalaCards integrated aliases for Dermatitis Herpetiformis:

Name: Dermatitis Herpetiformis 12 76 53 37 55 44 15 73
Duhring's Disease 12 76 53
Dermatosis Herpetiformis 12
Brocq-Duhring Disease 53
Duhring Brocq Disease 53
Dh 53

Classifications:



Summaries for Dermatitis Herpetiformis

NIH Rare Diseases : 53 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.

MalaCards based summary : Dermatitis Herpetiformis, also known as duhring's disease, is related to bullous pemphigoid and celiac disease 1. An important gene associated with Dermatitis Herpetiformis is TGM3 (Transglutaminase 3), and among its related pathways/superpathways are Cytokine Signaling in Immune system and Actin Nucleation by ARP-WASP Complex. The drugs Dapsone and leucovorin have been mentioned in the context of this disorder. Affiliated tissues include skin, small intestine and t cells, and related phenotypes are malabsorption and abnormal blistering of the skin

Wikipedia : 76 Dermatitis herpetiformis (DH) is a chronic blistering skin condition, characterised by blisters filled... more...

Related Diseases for Dermatitis Herpetiformis

Diseases in the Dermatitis Herpetiformis family:

Dermatitis Herpetiformis, Familial Juvenile Dermatitis Herpetiformis

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

(show top 50) (show all 181)
# Related Disease Score Top Affiliating Genes
1 bullous pemphigoid 32.8 CCL11 CD79A HLA-DQB1
2 celiac disease 1 30.2 CD79A HLA-A HLA-DQA1 HLA-DQB1 MYO9B TGM2
3 autoimmune disease 30.2 CD79A HLA-DQA1 HLA-DQB1
4 pityriasis rosea 30.0 HLA-A HLA-DQB1
5 immunoglobulin alpha deficiency 30.0 CD79A HLA-DQB1 TGM2
6 dermatitis, atopic 29.7 CCL11 IL31 TGM3
7 skin disease 29.7 CCL11 CD79A IL31 TGM3
8 dermatitis herpetiformis, familial 12.7
9 juvenile dermatitis herpetiformis 12.5
10 dehydrated hereditary stomatocytosis 1 with or without pseudohyperkalemia and/or perinatal edema 12.1
11 dermatitis 11.4
12 epilepsy occipital calcifications 11.3
13 dehydrated hereditary stomatocytosis 2 11.1
14 deafness-hypogonadism syndrome 11.1
15 lymphoma 10.6
16 lupus erythematosus 10.4
17 pemphigus 10.4
18 linear iga disease 10.4
19 ulcerative colitis 10.3
20 colitis 10.3
21 psoriasis 10.3
22 systemic lupus erythematosus 10.3
23 glomerulonephritis 10.3
24 arthritis 10.3
25 herpes gestationis 10.3
26 balanitis 10.2 TGM2 TGM3
27 balanitis xerotica obliterans 10.2 TGM2 TGM3
28 rheumatoid arthritis 10.2
29 urticaria 10.2
30 purpura 10.2
31 gastritis 10.2
32 cholangitis 10.2
33 discoid lupus erythematosus 10.2
34 autoimmune disease of skin and connective tissue 10.2 TGM2 TGM3
35 microscopic colitis 10.2 CD79A HLA-DQB1
36 strongyloidiasis 10.2 CCL11 CD79A
37 spinocerebellar ataxia 35 10.2 TGM2 TGM6
38 cryptosporidiosis 10.2 CCL11 CD79A
39 achalasia, familial esophageal 10.2 HLA-DQA1 HLA-DQB1
40 cholangitis, primary sclerosing 10.1
41 diabetes mellitus 10.1
42 erythema multiforme 10.1
43 protein-losing enteropathy 10.1
44 sclerosing cholangitis 10.1
45 epidermolysis bullosa 10.1
46 intestinal disease 10.1
47 thyroiditis 10.1
48 primary biliary cholangitis 10.1
49 depression 10.1
50 beryllium disease 10.1 HLA-DQA1 HLA-DQB1

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



Diseases related to Dermatitis Herpetiformis

Symptoms & Phenotypes for Dermatitis Herpetiformis

Human phenotypes related to Dermatitis Herpetiformis:

32 (show all 14)
# Description HPO Frequency HPO Source Accession
1 malabsorption 32 hallmark (90%) HP:0002024
2 abnormal blistering of the skin 32 hallmark (90%) HP:0008066
3 edema 32 occasional (7.5%) HP:0000969
4 pruritus 32 hallmark (90%) HP:0000989
5 autoimmunity 32 hallmark (90%) HP:0002960
6 microcytic anemia 32 hallmark (90%) HP:0001935
7 recurrent fractures 32 hallmark (90%) HP:0002757
8 erythema 32 hallmark (90%) HP:0010783
9 macule 32 hallmark (90%) HP:0012733
10 bone pain 32 occasional (7.5%) HP:0002653
11 eczema 32 frequent (33%) HP:0000964
12 urticaria 32 hallmark (90%) HP:0001025
13 skin vesicle 32 hallmark (90%) HP:0200037
14 lichenification 32 occasional (7.5%) HP:0100725

MGI Mouse Phenotypes related to Dermatitis Herpetiformis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.23 CCL11 CD79A HLA-DQA1 HLA-DQB1 MMP10 MMP12

Drugs & Therapeutics for Dermatitis Herpetiformis

Drugs for Dermatitis Herpetiformis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dapsone Approved, Investigational Phase 4 80-08-0 2955
2
leucovorin Approved Phase 4 58-05-9 6006 143
3
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
4 Vitamin B9 Phase 4
5 Folic Acid Antagonists Phase 4
6 Antiparasitic Agents Phase 4
7 Anti-Infective Agents Phase 4
8 Anti-Bacterial Agents Phase 4
9 Vitamin B Complex Phase 4
10 Antiprotozoal Agents Phase 4
11 Antimalarials Phase 4
12 Folate Phase 4
13 Antibodies Phase 1, Phase 2
14 Immunoglobulins Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Use of Dapsone Gel, 5% for Treating Dermatitis Herpetiformis Terminated NCT01115244 Phase 4 Dapsone gel, 5%
2 Study of Enzyme Supplements to Treat Celiac Disease Completed NCT00962182 Phase 1, Phase 2 STAN1;Placebo enzyme;STAN1+gluten
3 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Unknown status NCT02874079 Not Applicable
4 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275
5 Trichoscopy in Diagnosis of Immunobollous Diseases Not yet recruiting NCT03478072

Search NIH Clinical Center for Dermatitis Herpetiformis

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Cochrane evidence based reviews: dermatitis herpetiformis

Genetic Tests for Dermatitis Herpetiformis

Anatomical Context for Dermatitis Herpetiformis

MalaCards organs/tissues related to Dermatitis Herpetiformis:

41
Skin, Small Intestine, T Cells, Bone, Testes, Thyroid, B Cells

Publications for Dermatitis Herpetiformis

Articles related to Dermatitis Herpetiformis:

(show top 50) (show all 1020)
# Title Authors Year
1
Expression of Elafin in Dermatitis Herpetiformis. ( 29293478 )
2018
2
Why now? Delayed drug-induced pancreatitis due to dapsone for dermatitis herpetiformis. ( 29915658 )
2018
3
Novel association of a papuloerythroderma of Ofuji phenotype with dermatitis herpetiformis. ( 29664108 )
2018
4
Self-Reported Fractures in Dermatitis Herpetiformis Compared to Coeliac Disease. ( 29538319 )
2018
5
Prognosis of Dermatitis Herpetiformis Patients with and without Villous Atrophy at Diagnosis. ( 29783727 )
2018
6
Dermatitis, Herpetiformis ( 29630215 )
2018
7
Dermatitis Herpetiformis: A Common Extraintestinal Manifestation of Coeliac Disease. ( 29757210 )
2018
8
Monitoring of immunoglobulin A antibodies to epidermal and tissue transglutaminases over an 18-month period in a Japanese patient with dermatitis herpetiformis. ( 29464845 )
2018
9
Granular IgA Deposits in the Skin of Patients with Coeliac Disease: Is it Always Dermatitis Herpetiformis? ( 29972219 )
2018
10
Diagnostic Delay in Dermatitis Herpetiformis in a High-prevalence Area. ( 29048096 )
2018
11
A retrospective study of dermatitis herpetiformis from an immunobullous disease clinic in north India. ( 29752728 )
2018
12
Dermatitis herpetiformis in an African woman. ( 30364361 )
2018
13
Two Cases of Dermatitis Herpetiformis Successfully Treated with Tetracycline and Niacinamide. ( 30390734 )
2018
14
Coeliac disease and dermatitis herpetiformis. ( 30238885 )
2018
15
Coeliac disease and dermatitis herpetiformis. ( 30238886 )
2018
16
Coeliac disease and dermatitis herpetiformis - Authors' reply. ( 30238888 )
2018
17
Facial Involvement in Dermatitis Herpetiformis: A Case Report and Review of the Literature. ( 30103636 )
2018
18
Sensitivity of Transglutaminase 3 in the IgA Aggregates in Dermatitis Herpetiformis Skin to Potassium Iodide. ( 29571943 )
2018
19
Dermatitis herpetiformis and bone mineral density: analysis of a French cohort of 53 patients. ( 28524056 )
2017
20
Dapsone and sulfasalazine combination therapy in dermatitis herpetiformis. ( 28133723 )
2017
21
Immunoexpression of IgA receptors (CD89, CD71) in dermatitis herpetiformis. ( 29417981 )
2017
22
Dermatitis herpetiformis (Duhring's disease): a therapeutic challenge. ( 28497492 )
2017
23
Dermatitis herpetiformis presenting as pseudovasculitis. ( 28971130 )
2017
24
Dermatitis herpetiformis with fibrillar IgA deposition and unusual histologic findings. ( 28761917 )
2017
25
Dermatitis Herpetiformis as the Initial Presentation of Primary Biliary Cholangitis in a Male with Gluten Sensitivity. ( 28630805 )
2017
26
Serum levels and tissue expression of interleukin-31 in dermatitis herpetiformis and bullous pemphigoid. ( 28465041 )
2017
27
Expression of the JAK/STAT Signaling Pathway in Bullous Pemphigoid and Dermatitis Herpetiformis. ( 29203970 )
2017
28
The Role of Intereukin-31 in Pathogenesis of Itch and Its Intensity in a Course of Bullous Pemphigoid and Dermatitis Herpetiformis. ( 28808661 )
2017
29
Disappearance of epidermal transglutaminase and IgA deposits from the papillary dermis of dermatitis herpetiformis patients after a long-term gluten-free diet. ( 28906552 )
2017
30
Ex vivo Culture of Duodenal Biopsies from Patients with Dermatitis Herpetiformis Indicates that Transglutaminase 3 Antibody Production Occurs in the Gut. ( 29182792 )
2017
31
Exacerbation of Depression Symptoms in the Presence of Dermatitis Herpetiformis Rash, Celiac Disease, and Low Cholesterol. ( 29141126 )
2017
32
The HLA Alleles B*0801 and DRB1*0301 Are Associated with Dermatitis Herpetiformis in a Chinese Population. ( 26967484 )
2016
33
Topical Dapsone 5% Gel as an Effective Therapy in Dermatitis Herpetiformis. ( 27207357 )
2016
34
The frequency of mucosal-associated invariant T cells is selectively increased in dermatitis herpetiformis. ( 26940855 )
2016
35
Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis. ( 27983746 )
2016
36
Circulating Transglutaminase 3 - Immunoglobulin A immune complexes in dermatitis herpetiformis. ( 27106676 )
2016
37
Are anti-epidermal transglutaminase (eTG) antibodies titre correlated with dermatitis herpetiformis lesions during the disease follow-up? ( 27845514 )
2016
38
Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study. ( 27241271 )
2016
39
Autoimmunity to heat shock proteins and vitamin D status in patients with celiac disease without associated dermatitis herpetiformis. ( 27760369 )
2016
40
Decreased fibrinolytic potential and morphological changes of fibrin structure in dermatitis herpetiformis. ( 27440075 )
2016
41
Dermatitis Herpetiformis: A Review of Direct Immunofluorescence Findings. ( 26630684 )
2016
42
Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis. ( 28030659 )
2016
43
The Decreasing Prevalence of Severe Villous Atrophy in Dermatitis Herpetiformis: A 45-Year Experience in 393 Patients. ( 27136959 )
2016
44
Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. ( 27499257 )
2016
45
Pseudochromhidrosis: A rare case of yellowish discoloration of sweat in a patient with dermatitis herpetiformis and celiac disease. ( 29668179 )
2016
46
Acantholytic variant of Duhring's disease. ( 26765204 )
2016
47
Unique characteristics in Japanese dermatitis herpetiformis. ( 26119216 )
2015
48
The gluten-free diet and its current application in coeliac disease and dermatitis herpetiformis. ( 25922672 )
2015
49
The diagnosis and treatment of dermatitis herpetiformis. ( 25999753 )
2015
50
Serological marker of dermatitis herpetiformis in hypothyroidism due to Hashimoto's thyroiditis. ( 26693444 )
2015

Variations for Dermatitis Herpetiformis

Expression for Dermatitis Herpetiformis

Search GEO for disease gene expression data for Dermatitis Herpetiformis.

Pathways for Dermatitis Herpetiformis

GO Terms for Dermatitis Herpetiformis

Cellular components related to Dermatitis Herpetiformis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transport vesicle membrane GO:0030658 9.32 HLA-DQA1 HLA-DQB1
2 clathrin-coated endocytic vesicle membrane GO:0030669 9.26 HLA-DQA1 HLA-DQB1
3 MHC class II protein complex GO:0042613 9.16 HLA-DQA1 HLA-DQB1
4 ER to Golgi transport vesicle membrane GO:0012507 9.13 HLA-A HLA-DQA1 HLA-DQB1
5 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.8 HLA-A HLA-DQA1 HLA-DQB1

Biological processes related to Dermatitis Herpetiformis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.67 CD79A HLA-A HLA-DQA1 IL31
2 immune response GO:0006955 9.62 CCL11 HLA-A HLA-DQA1 HLA-DQB1
3 collagen catabolic process GO:0030574 9.4 MMP10 MMP12
4 interferon-gamma-mediated signaling pathway GO:0060333 9.33 HLA-A HLA-DQA1 HLA-DQB1
5 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.26 HLA-DQA1 HLA-DQB1
6 peptide cross-linking GO:0018149 9.13 TGM2 TGM3 TGM6
7 antigen processing and presentation GO:0019882 8.8 HLA-A HLA-DQA1 HLA-DQB1

Molecular functions related to Dermatitis Herpetiformis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transferase activity, transferring acyl groups GO:0016746 9.43 TGM2 TGM3 TGM6
2 MHC class II receptor activity GO:0032395 9.16 HLA-DQA1 HLA-DQB1
3 peptide antigen binding GO:0042605 9.13 HLA-A HLA-DQA1 HLA-DQB1
4 protein-glutamine gamma-glutamyltransferase activity GO:0003810 8.8 TGM2 TGM3 TGM6

Sources for Dermatitis Herpetiformis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
Content
Loading form....