DH
MCID: DRM007
MIFTS: 51

Dermatitis Herpetiformis (DH) malady

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

Aliases & Classifications for Dermatitis Herpetiformis

Aliases & Descriptions for Dermatitis Herpetiformis:

Name: Dermatitis Herpetiformis 12 50 52 42 14 69
Duhring's Disease 12 50
Dermatitis Herpetiformis [dup] 12
Dermatosis Herpetiformis 12
Brocq-Duhring Disease 50
Duhring Brocq Disease 50
Dh 50

Classifications:



External Ids:

Disease Ontology 12 DOID:8505
ICD10 33 L13.0
ICD9CM 35 694.0
MeSH 42 D003874
NCIt 47 C26742
UMLS 69 C0011608

Summaries for Dermatitis Herpetiformis

NIH Rare Diseases : 50 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 epidermolysis bullosa and colon adenocarcinoma, and has symptoms including pruritus, edema and macule. An important gene associated with Dermatitis Herpetiformis is CD79A (CD79a Molecule), and among its related pathways/superpathways are Allograft rejection and Cell adhesion molecules (CAMs). The drugs Immunoglobulins and Antibodies 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 : 71 Dermatitis herpetiformis (DH), or Duhring\'s disease, is a chronic blistering skin condition,... 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 219)
id Related Disease Score Top Affiliating Genes
1 epidermolysis bullosa 29.8 CCL11 CD79A IL4
2 colon adenocarcinoma 29.5 CCL11 IL4 TGM3
3 subcorneal pustular dermatosis 29.4 CD40LG ELN IL4
4 rheumatoid arthritis 29.2 CD40LG CD79A HLA-DQA1 HLA-DQB1 IL4
5 juvenile dermatitis herpetiformis 12.2
6 dermatitis herpetiformis, familial 12.2
7 dehydrated hereditary stomatocytosis with or without pseudohyperkalemia and/or perinatal edema 11.7
8 bullous pemphigoid 11.5
9 dermatitis 11.1
10 celiac disease 10.5
11 pyridoxine-responsive sideroblastic anemia 10.3 CD79A TGM2
12 cutaneous solitary mastocytoma 10.3 CD79A TGM2
13 amyotrophic lateral sclerosis type 10 10.3 CD79A TGM2
14 alcoholic neuropathy 10.3 CD40LG CD79A
15 camptodactyly vertebral fusion 10.3 CD40LG CD79A
16 spinal polio 10.3 CD40LG CD79A
17 rabies 10.3 CD40LG CD79A
18 unilateral retinoblastoma 10.3 CD40LG CD79A
19 mesangial proliferative glomerulonephritis 10.3 CD40LG CD79A
20 delta chain disease 10.3 CD40LG CD79A
21 discitis 10.3 CD40LG CD79A
22 spinal canal and spinal cord meningioma 10.3 CD40LG CD79A
23 trichostrongyloidiasis 10.3 CD40LG CD79A
24 colloid adenoma 10.3 CD40LG CD79A
25 heart septal defect 10.3 CD40LG CD79A
26 cutaneous polyarteritis nodosa 10.3 CD40LG CD79A
27 mild pre-eclampsia 10.3 CD40LG CD79A
28 retinal microaneurysm 10.3 CD40LG CD79A
29 pediatric meningioma 10.3 CD40LG CD79A
30 tonsil cancer 10.3 CD40LG CD79A
31 indomethacin antenatal infection 10.3 CD40LG CD79A
32 postcricoid region cancer 10.3 CD40LG CD79A
33 beach ear 10.3 CD40LG CD79A
34 acute kidney tubular necrosis 10.3 CD40LG CD79A
35 atheroembolism of kidney 10.3 CD40LG CD79A
36 partial sensory epilepsy 10.3 CD40LG CD79A
37 ritscher-schinzel syndrome 1 10.3 CD40LG CD79A
38 endocarditis 10.3 CD40LG CD79A
39 muscular dystrophy 10.3 CD40LG CD79A
40 dumping syndrome 10.3 CD40LG CD79A
41 striatonigral degeneration 10.3 CD40LG CD79A
42 ciliary body spindle cell melanoma 10.3 CD40LG CD79A
43 purulent endophthalmitis 10.3 CD40LG CD79A
44 mu chain disease 10.3 CD40LG CD79A
45 generalized atherosclerosis 10.3 CD40LG CD79A
46 bursitis 10.3 CD40LG CD79A
47 syphilis 10.3 CD40LG CD79A
48 homocystinuria 10.3 CD40LG CD79A
49 mental retardation, autosomal dominant 19 10.3 CD40LG CD79A
50 hemangioma of orbit 10.3 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)
id Description HPO Frequency HPO Source Accession
1 pruritus 32 HP:0000989
2 edema 32 HP:0000969
3 macule 32 HP:0012733
4 bone pain 32 HP:0002653
5 malabsorption 32 HP:0002024
6 abnormal blistering of the skin 32 HP:0008066
7 autoimmunity 32 HP:0002960
8 microcytic anemia 32 HP:0001935
9 recurrent fractures 32 HP:0002757
10 erythema 32 HP:0010783
11 eczema 32 HP:0000964
12 urticaria 32 HP:0001025
13 skin vesicle 32 HP:0200037
14 lichenification 32 HP:0100725

MGI Mouse Phenotypes related to Dermatitis Herpetiformis:

44
id 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 HLA-DQA1 HLA-DQB1 IL4 MMP12 MYO9B TGM2
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)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins Phase 1, Phase 2
2 Antibodies Phase 1, Phase 2
3
Dapsone Approved, Investigational 80-08-0 2955
4
leucovorin Approved, Nutraceutical 58-05-9 54575, 6560146 143
5
Folic Acid Approved, Nutraceutical, Vet_approved 59-30-3 6037
6 Folic Acid Antagonists
7 Vitamin B Complex
8 Anti-Bacterial Agents
9 Anti-Infective Agents
10 Antimalarials
11 Antiparasitic Agents
12 Antiprotozoal Agents
13 Folate Nutraceutical
14 Vitamin B9 Nutraceutical

Interventional clinical trials:


id Name Status NCT ID Phase
1 Study of Enzyme Supplements to Treat Celiac Disease Active, not recruiting NCT00962182 Phase 1, Phase 2
2 Use of Dapsone Gel, 5% for Treating Dermatitis Herpetiformis Unknown status NCT01115244
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 48 :


Cochrane evidence based reviews: dermatitis herpetiformis

Genetic Tests for Dermatitis Herpetiformis

Anatomical Context for Dermatitis Herpetiformis

MalaCards organs/tissues related to Dermatitis Herpetiformis:

39
Skin, T Cells, Bone, Thyroid, Testes, Small Intestine, Endothelial

Publications for Dermatitis Herpetiformis

Articles related to Dermatitis Herpetiformis:

(show top 50) (show all 587)
id Title Authors Year
1
Serum levels and tissue expression of interleukin-31 in dermatitis herpetiformis and bullous pemphigoid. ( 28465041 )
2017
2
Dermatitis herpetiformis and bone mineral density: analysis of a French cohort of 53 patients. ( 28524056 )
2017
3
Increased Serum Levels of Th2-type Cytokines and Eotaxin in Fibrillar-type Dermatitis Herpetiformis. ( 27983746 )
2016
4
The frequency of mucosal-associated invariant T cells is selectively increased in dermatitis herpetiformis. ( 26940855 )
2016
5
Circulating Transglutaminase 3 - Immunoglobulin A immune complexes in dermatitis herpetiformis. ( 27106676 )
2016
6
Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. ( 27499257 )
2016
7
Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis. ( 28030659 )
2016
8
Are anti-epidermal transglutaminase (eTG) antibodies titre correlated with dermatitis herpetiformis lesions during the disease follow-up? ( 27845514 )
2016
9
Dermatitis Herpetiformis: A Review of Direct Immunofluorescence Findings. ( 26630684 )
2016
10
Autoimmunity to heat shock proteins and vitamin D status in patients with celiac disease without associated dermatitis herpetiformis. ( 27760369 )
2016
11
The Decreasing Prevalence of Severe Villous Atrophy in Dermatitis Herpetiformis: A 45-Year Experience in 393 Patients. ( 27136959 )
2016
12
Decreased fibrinolytic potential and morphological changes of fibrin structure in dermatitis herpetiformis. ( 27440075 )
2016
13
Topical Dapsone 5% Gel as an Effective Therapy in Dermatitis Herpetiformis. ( 27207357 )
2016
14
The HLA Alleles B*0801 and DRB1*0301 Are Associated with Dermatitis Herpetiformis in a Chinese Population. ( 26967484 )
2016
15
Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study. ( 27241271 )
2016
16
Celiac disease evolving into dermatitis herpetiformis in patients adhering to normal or gluten-free diet. ( 25639558 )
2015
17
The diagnosis and treatment of dermatitis herpetiformis. ( 25999753 )
2015
18
Utility of Direct Immunofluorescence Testing for IgA in Patients With High and Low Clinical Suspicion for Dermatitis Herpetiformis. ( 26572994 )
2015
19
A rare bullous variant of dermatitis herpetiformis. ( 26042658 )
2015
20
Dermatitis Herpetiformis Refractory to Gluten-free Dietary Treatment. ( 26084552 )
2015
21
Unique characteristics in Japanese dermatitis herpetiformis. ( 26119216 )
2015
22
Regulatory T cells as well as IL-10 are reduced in the skin of patients with dermatitis herpetiformis. ( 25465638 )
2015
23
Serological marker of dermatitis herpetiformis in hypothyroidism due to Hashimoto's thyroiditis. ( 26693444 )
2015
24
Dermatitis Herpetiformis: Pathognomonic Transglutaminase IgA Deposits in the Skin and Excellent Prognosis on a Gluten-free Diet. ( 26059085 )
2015
25
Tissue Factor in Dermatitis Herpetiformis and Bullous Pemphigoid: Link between Immune and Coagulation System in Subepidermal Autoimmune Bullous Diseases. ( 27057091 )
2015
26
The gluten-free diet and its current application in coeliac disease and dermatitis herpetiformis. ( 25922672 )
2015
27
Quality of Life and Gastrointestinal Symptoms in Long-Term Treated Dermatitis Herpetiformis Patients: A Cross-Sectional Study in Finland. ( 26267424 )
2015
28
Dermatitis herpetiformis: clinical presentations are independent of manifestations of celiac disease. ( 24293087 )
2014
29
Iga anti-epidermal transglutaminase autoantibodies: a simple test to improve differential diagnosis between dermatitis herpetiformis and atopic dermatitis. ( 25502537 )
2014
30
Persistent pruritic papulovesicles. Dermatitis herpetiformis. ( 25251235 )
2014
31
High prevalence of cryofibrinogenaemia in dermatitis herpetiformis. ( 25490996 )
2014
32
Skin manifestations of celiac disease: not always dermatitis herpetiformis. ( 24601945 )
2014
33
Petechial eruption on fingers. Dermatitis herpetiformis. ( 25317570 )
2014
34
Erythema elevatum diutinum in association with dermatitis herpetiformis. ( 24616856 )
2014
35
Evidence for a role of autoantibodies to heat shock protein 60, 70, and 90 in patients with dermatitis herpetiformis. ( 24643797 )
2014
36
Dermatitis herpetiformis. ( 24740905 )
2014
37
Letter to editor about dermatitis herpetiformis, the celiac disease of the skin! ( 25003271 )
2014
38
Dermatitis herpetiformis in children: a long-term follow-up study. ( 24720674 )
2014
39
Mediators of mast cells in bullous pemphigoid and dermatitis herpetiformis. ( 25400334 )
2014
40
Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment. ( 25387490 )
2014
41
A case of dermatitis herpetiformis after a mini-gastric bypass surgery. ( 24947705 )
2014
42
Dermatitis herpetiformis. ( 25526342 )
2014
43
Dermatitis herpetiformis in Brazilan male celiac disease patients: A case series. ( 25544421 )
2014
44
A response from the authors of An unusual case of dermatitis herpetiformis:. ( 25039709 )
2014
45
Dermatitis herpetiformis: not only in adults. ( 25039708 )
2014
46
IgA anti-epidermal transglutaminase antibodies in dermatitis herpetiformis: a significant but not complete response to a gluten-free diet treatment. ( 25196300 )
2014
47
Incidence and prevalence of celiac disease and dermatitis herpetiformis in the UK over two decades: population-based study. ( 24667576 )
2014
48
Response to letter to the editor on skin manifestations of celiac disease: not always dermatitis herpetiformis. ( 24899090 )
2014
49
A case of dermatitis herpetiformis with blister formation between laminin-332 and type 7 collagen. ( 25297981 )
2014
50
Transglutaminase 3 Present in the IgA Aggregates in Dermatitis Herpetiformis Skin Is Enzymatically Active and Binds Soluble Fibrinogen. ( 25178107 )
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:

id 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:

id 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 9.4 CD79A IL4
3 positive regulation of interleukin-10 production GO:0032733 9.37 CD40LG IL4
4 immune response GO:0006955 9.35 CCL11 CD40LG HLA-DQA1 HLA-DQB1 IL4
5 B cell differentiation GO:0030183 9.33 CD40LG CD79A IL4
6 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.32 HLA-DQA1 HLA-DQB1
7 peptide cross-linking GO:0018149 8.8 TGM2 TGM3 TGM6

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

id 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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