MCID: DRM007
MIFTS: 56

Dermatitis Herpetiformis

Categories: Rare diseases, Skin diseases, Immune diseases, Genetic 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 dermatitis. An important gene associated with Dermatitis Herpetiformis is CD79A (CD79a Molecule), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Dapsone and leucovorin have been mentioned in the context of this disorder. Affiliated tissues include skin, t cells and bone, and related phenotypes are eczema and edema

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 289)
# Related Disease Score Top Affiliating Genes
1 bullous pemphigoid 33.1 CCL11 CD79A HLA-DQB1
2 dermatitis 31.0 CCL11 IL31 IL4
3 erythema elevatum diutinum 31.0 CD40LG CD79A
4 immunoglobulin alpha deficiency 29.8 CD40LG CD79A HLA-DQB1 TGM2
5 systemic lupus erythematosus 28.8 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
6 autoimmune disease 28.7 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
7 rheumatoid arthritis 28.7 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
8 skin disease 28.7 CD79A IL31 IL4
9 dermatitis, atopic 28.6 CCL11 IL31 IL4 TGM3
10 celiac disease 1 28.2 CD40LG CD79A HLA-DQA1 HLA-DQB1 MYO9B TGM2
11 dermatitis herpetiformis, familial 12.5
12 juvenile dermatitis herpetiformis 12.3
13 dehydrated hereditary stomatocytosis 1 with or without pseudohyperkalemia and/or perinatal edema 11.9
14 epilepsy occipital calcifications 11.1
15 gluten allergy 10.9 CD79A TGM2
16 balanitis 10.9 TGM2 TGM3
17 wheat allergy 10.9 CD79A TGM2
18 balanitis xerotica obliterans 10.8 TGM2 TGM3
19 selective igg deficiency disease 10.8 CD40LG CD79A
20 c1q nephropathy 10.8 CD40LG CD79A
21 meningovascular neurosyphilis 10.8 CD40LG CD79A
22 alpha chain disease 10.8 CD40LG CD79A
23 brill-zinsser disease 10.8 CD40LG CD79A
24 exudative glomerulonephritis 10.8 CD40LG CD79A
25 cork-handlers' disease 10.8 CD40LG CD79A
26 salpingo-oophoritis 10.8 CD40LG CD79A
27 early yaws 10.8 CD40LG CD79A
28 ventilation pneumonitis 10.8 CD40LG CD79A
29 chronic interstitial cystitis 10.8 CD40LG CD79A
30 axillary adenitis 10.8 CD40LG CD79A
31 cryofibrinogenemia 10.8 CD40LG CD79A
32 trichostrongyloidiasis 10.8 CD40LG CD79A
33 subacute bacterial endocarditis 10.8 CD40LG CD79A
34 heterophyiasis 10.8 CD40LG CD79A
35 immunoglobulin g deficiency 10.8 CD40LG CD79A
36 orbital granuloma 10.8 CD40LG CD79A
37 transient hypogammaglobulinemia of infancy 10.8 CD40LG CD79A
38 hyperglobulinemic purpura 10.8 CD40LG CD79A
39 gastroduodenitis 10.8 CD40LG CD79A
40 bacterial conjunctivitis 10.8 CD40LG CD79A
41 heavy chain disease 10.8 CD40LG CD79A
42 ophthalmia neonatorum 10.8 CD40LG CD79A
43 parotid disease 10.8 CD40LG CD79A
44 cerebral arteritis 10.8 CD40LG CD79A
45 selective immunoglobulin deficiency disease 10.8 CD40LG CD79A
46 geniculate herpes zoster 10.8 CD40LG CD79A
47 strongyloidiasis 10.8 CCL11 CD79A
48 sporotrichosis 10.8 CD40LG CD79A
49 fallopian tube disease 10.8 CD40LG CD79A
50 hyperimmunoglobulin syndrome 10.8 CD40LG CD79A

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

MGI Mouse Phenotypes related to Dermatitis Herpetiformis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.81 CCL11 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
2 immune system MP:0005387 9.61 CCL11 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
3 renal/urinary system MP:0005367 9.1 TGM3 CD40LG CD79A HLA-DQB1 IL4 TGM2

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, Nutraceutical Phase 4 58-05-9 143 6006
3
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
4 Folic Acid Antagonists Phase 4
5 Anti-Bacterial Agents Phase 4
6 Vitamin B Complex Phase 4
7 Anti-Infective Agents Phase 4
8 Antiparasitic Agents Phase 4
9 Antiprotozoal Agents Phase 4
10 Antimalarials Phase 4
11 Folate Nutraceutical Phase 4
12 Vitamin B9 Nutraceutical 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 Recruiting 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, T Cells, Bone, Thyroid, Small Intestine, Neutrophil, Testes

Publications for Dermatitis Herpetiformis

Articles related to Dermatitis Herpetiformis:

(show top 50) (show all 613)
# 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
Sensitivity of Transglutaminase 3 in the IgA Aggregates in Dermatitis Herpetiformis Skin to Potassium Iodide. ( 29571943 )
2018
13
Dermatitis herpetiformis and bone mineral density: analysis of a French cohort of 53 patients. ( 28524056 )
2017
14
Dapsone and sulfasalazine combination therapy in dermatitis herpetiformis. ( 28133723 )
2017
15
Immunoexpression of IgA receptors (CD89, CD71) in dermatitis herpetiformis. ( 29417981 )
2017
16
Dermatitis herpetiformis (Duhring's disease): a therapeutic challenge. ( 28497492 )
2017
17
Dermatitis herpetiformis presenting as pseudovasculitis. ( 28971130 )
2017
18
Dermatitis herpetiformis with fibrillar IgA deposition and unusual histologic findings. ( 28761917 )
2017
19
Dermatitis Herpetiformis as the Initial Presentation of Primary Biliary Cholangitis in a Male with Gluten Sensitivity. ( 28630805 )
2017
20
Serum levels and tissue expression of interleukin-31 in dermatitis herpetiformis and bullous pemphigoid. ( 28465041 )
2017
21
Expression of the JAK/STAT Signaling Pathway in Bullous Pemphigoid and Dermatitis Herpetiformis. ( 29203970 )
2017
22
The Role of Intereukin-31 in Pathogenesis of Itch and Its Intensity in a Course of Bullous Pemphigoid and Dermatitis Herpetiformis. ( 28808661 )
2017
23
Disappearance of epidermal transglutaminase and IgA deposits from the papillary dermis of dermatitis herpetiformis patients after a long-term gluten-free diet. ( 28906552 )
2017
24
Ex vivo Culture of Duodenal Biopsies from Patients with Dermatitis Herpetiformis Indicates that Transglutaminase 3 Antibody Production Occurs in the Gut. ( 29182792 )
2017
25
Exacerbation of Depression Symptoms in the Presence of Dermatitis Herpetiformis Rash, Celiac Disease, and Low Cholesterol. ( 29141126 )
2017
26
The HLA Alleles B*0801 and DRB1*0301 Are Associated with Dermatitis Herpetiformis in a Chinese Population. ( 26967484 )
2016
27
Topical Dapsone 5% Gel as an Effective Therapy in Dermatitis Herpetiformis. ( 27207357 )
2016
28
The frequency of mucosal-associated invariant T cells is selectively increased in dermatitis herpetiformis. ( 26940855 )
2016
29
Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis. ( 27983746 )
2016
30
Circulating Transglutaminase 3 - Immunoglobulin A immune complexes in dermatitis herpetiformis. ( 27106676 )
2016
31
Are anti-epidermal transglutaminase (eTG) antibodies titre correlated with dermatitis herpetiformis lesions during the disease follow-up? ( 27845514 )
2016
32
Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study. ( 27241271 )
2016
33
Autoimmunity to heat shock proteins and vitamin D status in patients with celiac disease without associated dermatitis herpetiformis. ( 27760369 )
2016
34
Decreased fibrinolytic potential and morphological changes of fibrin structure in dermatitis herpetiformis. ( 27440075 )
2016
35
Dermatitis Herpetiformis: A Review of Direct Immunofluorescence Findings. ( 26630684 )
2016
36
Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis. ( 28030659 )
2016
37
The Decreasing Prevalence of Severe Villous Atrophy in Dermatitis Herpetiformis: A 45-Year Experience in 393 Patients. ( 27136959 )
2016
38
Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. ( 27499257 )
2016
39
Unique characteristics in Japanese dermatitis herpetiformis. ( 26119216 )
2015
40
The gluten-free diet and its current application in coeliac disease and dermatitis herpetiformis. ( 25922672 )
2015
41
The diagnosis and treatment of dermatitis herpetiformis. ( 25999753 )
2015
42
Serological marker of dermatitis herpetiformis in hypothyroidism due to Hashimoto's thyroiditis. ( 26693444 )
2015
43
Regulatory T cells as well as IL-10 are reduced in the skin of patients with dermatitis herpetiformis. ( 25465638 )
2015
44
Dermatitis Herpetiformis: Pathognomonic Transglutaminase IgA Deposits in the Skin and Excellent Prognosis on a Gluten-free Diet. ( 26059085 )
2015
45
Celiac disease evolving into dermatitis herpetiformis in patients adhering to normal or gluten-free diet. ( 25639558 )
2015
46
Dermatitis Herpetiformis Refractory to Gluten-free Dietary Treatment. ( 26084552 )
2015
47
Utility of Direct Immunofluorescence Testing for IgA in Patients With High and Low Clinical Suspicion for Dermatitis Herpetiformis. ( 26572994 )
2015
48
A rare bullous variant of dermatitis herpetiformis. ( 26042658 )
2015
49
Quality of Life and Gastrointestinal Symptoms in Long-Term Treated Dermatitis Herpetiformis Patients: A Cross-Sectional Study in Finland. ( 26267424 )
2015
50
Tissue Factor in Dermatitis Herpetiformis and Bullous Pemphigoid: Link between Immune and Coagulation System in Subepidermal Autoimmune Bullous Diseases. ( 27057091 )
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 clathrin-coated endocytic vesicle membrane GO:0030669 9.16 HLA-DQA1 HLA-DQB1
2 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DQA1 HLA-DQB1
3 MHC class II protein complex GO:0042613 8.62 HLA-DQA1 HLA-DQB1

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

# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.67 CCL11 CD40LG IL31 IL4
2 B cell proliferation GO:0042100 9.43 CD40LG CD79A
3 T cell costimulation GO:0031295 9.43 CD40LG HLA-DQA1 HLA-DQB1
4 B cell activation GO:0042113 9.4 CD79A IL4
5 immune response GO:0006955 9.35 CCL11 CD40LG HLA-DQA1 HLA-DQB1 IL4
6 B cell differentiation GO:0030183 9.33 CD40LG CD79A IL4
7 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.32 HLA-DQA1 HLA-DQB1
8 peptide cross-linking GO:0018149 8.8 TGM2 TGM3 TGM6

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 peptide antigen binding GO:0042605 9.37 HLA-DQA1 HLA-DQB1
3 cytokine receptor binding GO:0005126 9.32 IL31 IL4
4 cytokine activity GO:0005125 9.26 CCL11 CD40LG IL31 IL4
5 MHC class II receptor activity GO:0032395 9.16 HLA-DQA1 HLA-DQB1
6 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
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