MCID: DRM007
MIFTS: 56

Dermatitis Herpetiformis

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

Aliases & Classifications for Dermatitis Herpetiformis

MalaCards integrated aliases for Dermatitis Herpetiformis:

Name: Dermatitis Herpetiformis 12 72 49 36 51 41 14 69
Duhring's Disease 12 72 49
Dermatitis Herpetiformis [dup] 12
Dermatosis Herpetiformis 12
Brocq-Duhring Disease 49
Duhring Brocq Disease 49
Dh 49

Classifications:



Summaries for Dermatitis Herpetiformis

NIH Rare Diseases : 49 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 bullous pemphigoid and erythema elevatum diutinum, and has symptoms including eczema, edema and pruritus. An important gene associated with Dermatitis Herpetiformis is CD79A (CD79a Molecule), and among its related pathways/superpathways are Allograft rejection and G-protein signaling Regulation of p38 and JNK signaling mediated by G-proteins. 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 hematopoietic system and immune system

Wikipedia : 72 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 288)
# Related Disease Score Top Affiliating Genes
1 bullous pemphigoid 32.9 CCL11 CD79A HLA-DQB1
2 erythema elevatum diutinum 30.6 CD40LG CD79A
3 immunoglobulin alpha deficiency 29.7 CD40LG CD79A HLA-DQB1 TGM2
4 bone inflammation disease 29.5 CD40LG CD79A IL4
5 dermatitis, atopic 29.4 CCL11 IL4 TGM3
6 celiac disease 1 29.2 CD40LG CD79A HLA-DQA1 HLA-DQB1 MYO9B TGM2
7 autoimmune disease 28.8 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
8 rheumatoid arthritis 28.8 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
9 systemic lupus erythematosus 28.7 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
10 dermatitis herpetiformis, familial 12.5
11 juvenile dermatitis herpetiformis 12.3
12 dehydrated hereditary stomatocytosis 1 with or without pseudohyperkalemia and/or perinatal edema 11.9
13 dermatitis 11.2
14 epilepsy occipital calcifications 11.1
15 gluten allergy 10.6 CD79A TGM2
16 balanitis 10.5 TGM2 TGM3
17 wheat allergy 10.5 CD79A TGM2
18 selective igg deficiency disease 10.5 CD40LG CD79A
19 c1q nephropathy 10.5 CD40LG CD79A
20 balanitis xerotica obliterans 10.5 TGM2 TGM3
21 meningovascular neurosyphilis 10.5 CD40LG CD79A
22 alpha chain disease 10.5 CD40LG CD79A
23 brill-zinsser disease 10.5 CD40LG CD79A
24 exudative glomerulonephritis 10.5 CD40LG CD79A
25 cork-handlers' disease 10.5 CD40LG CD79A
26 salpingo-oophoritis 10.5 CD40LG CD79A
27 early yaws 10.5 CD40LG CD79A
28 ventilation pneumonitis 10.5 CD40LG CD79A
29 chronic interstitial cystitis 10.5 CD40LG CD79A
30 axillary adenitis 10.5 CD40LG CD79A
31 cryofibrinogenemia 10.5 CD40LG CD79A
32 trichostrongyloidiasis 10.5 CD40LG CD79A
33 subacute bacterial endocarditis 10.5 CD40LG CD79A
34 heterophyiasis 10.5 CD40LG CD79A
35 immunoglobulin g deficiency 10.5 CD40LG CD79A
36 orbital granuloma 10.5 CD40LG CD79A
37 transient hypogammaglobulinemia of infancy 10.5 CD40LG CD79A
38 hyperglobulinemic purpura 10.5 CD40LG CD79A
39 gastroduodenitis 10.5 CD40LG CD79A
40 bacterial conjunctivitis 10.5 CD40LG CD79A
41 heavy chain disease 10.5 CD40LG CD79A
42 ophthalmia neonatorum 10.5 CD40LG CD79A
43 parotid disease 10.5 CD40LG CD79A
44 cerebral arteritis 10.5 CD40LG CD79A
45 selective immunoglobulin deficiency disease 10.5 CD40LG CD79A
46 geniculate herpes zoster 10.5 CD40LG CD79A
47 sporotrichosis 10.5 CD40LG CD79A
48 fallopian tube disease 10.5 CD40LG CD79A
49 strongyloidiasis 10.5 CCL11 CD79A
50 hyperimmunoglobulin syndrome 10.5 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:

31 (show all 14)
# Description HPO Frequency HPO Source Accession
1 eczema 31 frequent (33%) HP:0000964
2 edema 31 occasional (7.5%) HP:0000969
3 pruritus 31 hallmark (90%) HP:0000989
4 urticaria 31 hallmark (90%) HP:0001025
5 microcytic anemia 31 hallmark (90%) HP:0001935
6 malabsorption 31 hallmark (90%) HP:0002024
7 bone pain 31 occasional (7.5%) HP:0002653
8 recurrent fractures 31 hallmark (90%) HP:0002757
9 autoimmunity 31 hallmark (90%) HP:0002960
10 abnormal blistering of the skin 31 hallmark (90%) HP:0008066
11 erythema 31 hallmark (90%) HP:0010783
12 macule 31 hallmark (90%) HP:0012733
13 lichenification 31 occasional (7.5%) HP:0100725
14 skin vesicle 31 hallmark (90%) HP:0200037

MGI Mouse Phenotypes related to Dermatitis Herpetiformis:

43
# 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 CD40LG CD79A HLA-DQB1 IL4 TGM2 TGM3

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 Vitamin B Complex Phase 4
6 Anti-Bacterial Agents Phase 4
7 Anti-Infective Agents Phase 4
8 Antimalarials Phase 4
9 Antiprotozoal Agents Phase 4
10 Antiparasitic Agents Phase 4
11 Folate Nutraceutical Phase 4
12 Vitamin B9 Nutraceutical Phase 4
13 Immunoglobulins Phase 1, Phase 2
14 Antibodies 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 Unknown status NCT00962182 Phase 1, Phase 2 STAN1;Placebo enzyme;STAN1+gluten
3 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Recruiting NCT02874079
4 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275

Search NIH Clinical Center for Dermatitis Herpetiformis

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cochrane evidence based reviews: dermatitis herpetiformis

Genetic Tests for Dermatitis Herpetiformis

Anatomical Context for Dermatitis Herpetiformis

MalaCards organs/tissues related to Dermatitis Herpetiformis:

38
Skin, T Cells, Bone, Thyroid, Neutrophil, Small Intestine, Testes

Publications for Dermatitis Herpetiformis

Articles related to Dermatitis Herpetiformis:

(show top 50) (show all 600)
# Title Authors Year
1
Expression of Elafin in Dermatitis Herpetiformis. ( 29293478 )
2018
2
Diagnostic Delay in Dermatitis Herpetiformis in a High-prevalence Area. ( 29048096 )
2018
3
Immunoexpression of IgA receptors (CD89, CD71) in dermatitis herpetiformis. ( 29417981 )
2017
4
Serum levels and tissue expression of interleukin-31 in dermatitis herpetiformis and bullous pemphigoid. ( 28465041 )
2017
5
Dermatitis herpetiformis with fibrillar IgA deposition and unusual histologic findings. ( 28761917 )
2017
6
Dermatitis herpetiformis and bone mineral density: analysis of a French cohort of 53 patients. ( 28524056 )
2017
7
Expression of the JAK/STAT Signaling Pathway in Bullous Pemphigoid and Dermatitis Herpetiformis. ( 29203970 )
2017
8
The Role of Intereukin-31 in Pathogenesis of Itch and Its Intensity in a Course of Bullous Pemphigoid and Dermatitis Herpetiformis. ( 28808661 )
2017
9
Exacerbation of Depression Symptoms in the Presence of Dermatitis Herpetiformis Rash, Celiac Disease, and Low Cholesterol. ( 29141126 )
2017
10
Dermatitis herpetiformis (Duhring's disease): a therapeutic challenge. ( 28497492 )
2017
11
Dermatitis herpetiformis presenting as pseudovasculitis. ( 28971130 )
2017
12
Dermatitis Herpetiformis as the Initial Presentation of Primary Biliary Cholangitis in a Male with Gluten Sensitivity. ( 28630805 )
2017
13
Dapsone and sulfasalazine combination therapy in dermatitis herpetiformis. ( 28133723 )
2017
14
Disappearance of epidermal transglutaminase and IgA deposits from the papillary dermis of dermatitis herpetiformis patients after a long-term gluten-free diet. ( 28906552 )
2017
15
Ex vivo Culture of Duodenal Biopsies from Patients with Dermatitis Herpetiformis Indicates that Transglutaminase 3 Antibody Production Occurs in the Gut. ( 29182792 )
2017
16
Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis. ( 28030659 )
2016
17
Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study. ( 27241271 )
2016
18
The HLA Alleles B*0801 and DRB1*0301 Are Associated with Dermatitis Herpetiformis in a Chinese Population. ( 26967484 )
2016
19
The frequency of mucosal-associated invariant T cells is selectively increased in dermatitis herpetiformis. ( 26940855 )
2016
20
Circulating Transglutaminase 3 - Immunoglobulin A immune complexes in dermatitis herpetiformis. ( 27106676 )
2016
21
Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis. ( 27983746 )
2016
22
Decreased fibrinolytic potential and morphological changes of fibrin structure in dermatitis herpetiformis. ( 27440075 )
2016
23
Autoimmunity to heat shock proteins and vitamin D status in patients with celiac disease without associated dermatitis herpetiformis. ( 27760369 )
2016
24
Topical Dapsone 5% Gel as an Effective Therapy in Dermatitis Herpetiformis. ( 27207357 )
2016
25
Dermatitis Herpetiformis: A Review of Direct Immunofluorescence Findings. ( 26630684 )
2016
26
The Decreasing Prevalence of Severe Villous Atrophy in Dermatitis Herpetiformis: A 45-Year Experience in 393 Patients. ( 27136959 )
2016
27
Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. ( 27499257 )
2016
28
Are anti-epidermal transglutaminase (eTG) antibodies titre correlated with dermatitis herpetiformis lesions during the disease follow-up? ( 27845514 )
2016
29
Dermatitis Herpetiformis Refractory to Gluten-free Dietary Treatment. ( 26084552 )
2015
30
A rare bullous variant of dermatitis herpetiformis. ( 26042658 )
2015
31
Unique characteristics in Japanese dermatitis herpetiformis. ( 26119216 )
2015
32
Tissue Factor in Dermatitis Herpetiformis and Bullous Pemphigoid: Link between Immune and Coagulation System in Subepidermal Autoimmune Bullous Diseases. ( 27057091 )
2015
33
Quality of Life and Gastrointestinal Symptoms in Long-Term Treated Dermatitis Herpetiformis Patients: A Cross-Sectional Study in Finland. ( 26267424 )
2015
34
Dermatitis Herpetiformis: Pathognomonic Transglutaminase IgA Deposits in the Skin and Excellent Prognosis on a Gluten-free Diet. ( 26059085 )
2015
35
Serological marker of dermatitis herpetiformis in hypothyroidism due to Hashimoto's thyroiditis. ( 26693444 )
2015
36
The gluten-free diet and its current application in coeliac disease and dermatitis herpetiformis. ( 25922672 )
2015
37
Utility of Direct Immunofluorescence Testing for IgA in Patients With High and Low Clinical Suspicion for Dermatitis Herpetiformis. ( 26572994 )
2015
38
The diagnosis and treatment of dermatitis herpetiformis. ( 25999753 )
2015
39
Regulatory T cells as well as IL-10 are reduced in the skin of patients with dermatitis herpetiformis. ( 25465638 )
2015
40
Celiac disease evolving into dermatitis herpetiformis in patients adhering to normal or gluten-free diet. ( 25639558 )
2015
41
Skin manifestations of celiac disease: not always dermatitis herpetiformis. ( 24601945 )
2014
42
Transglutaminase 3 Present in the IgA Aggregates in Dermatitis Herpetiformis Skin Is Enzymatically Active and Binds Soluble Fibrinogen. ( 25178107 )
2014
43
Dermatitis herpetiformis. ( 25526342 )
2014
44
Petechial eruption on fingers. Dermatitis herpetiformis. ( 25317570 )
2014
45
A case of dermatitis herpetiformis with blister formation between laminin-332 and type 7 collagen. ( 25297981 )
2014
46
Erythema elevatum diutinum in association with dermatitis herpetiformis. ( 24616856 )
2014
47
Letter to editor about dermatitis herpetiformis, the celiac disease of the skin! ( 25003271 )
2014
48
Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment. ( 25387490 )
2014
49
Mediators of mast cells in bullous pemphigoid and dermatitis herpetiformis. ( 25400334 )
2014
50
IgA anti-epidermal transglutaminase antibodies in dermatitis herpetiformis: a significant but not complete response to a gluten-free diet treatment. ( 25196300 )
2014

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 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DQA1 HLA-DQB1
2 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 T cell costimulation GO:0031295 9.43 CD40LG HLA-DQA1 HLA-DQB1
2 B cell activation GO:0042113 9.37 CD79A IL4
3 immune response GO:0006955 9.35 CCL11 CD40LG HLA-DQA1 HLA-DQB1 IL4
4 B cell differentiation GO:0030183 9.33 CD40LG CD79A IL4
5 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.32 HLA-DQA1 HLA-DQB1
6 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 9.33 TGM2 TGM3 TGM6
2 peptide antigen binding GO:0042605 9.26 HLA-DQA1 HLA-DQB1
3 MHC class II receptor activity GO:0032395 8.96 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
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....