Details of Disease

General Information of Disease (ID: DIS0TC0G)

Disease Name	Dermatitis herpetiformis
Synonyms	dermatosis herpetiformis; Duhring's disease; dermatitis herpetiformis; Durhing-Brocq disease
Disease Class	EB44: Dermatitis herpetiformis
Definition	Dermatitis herpetiformis (DH) is a chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. DH may also appear as a consequence of gluten intolerance.
Disease Hierarchy	DIS3AYL6: Autoimmune bullous skin disease DIS0TC0G: Dermatitis herpetiformis
ICD Code	ICD-11 ICD-11: EB44 ICD-10 ICD-10: L13.0 ICD-9 ICD-9: 694 Expand ICD-11 'EB44 Expand ICD-10 'L13.0 Expand ICD-9 694
Disease Identifiers	MONDO ID MONDO_0015614 MESH ID D003874 UMLS CUI C0011608 MedGen ID 8327 Orphanet ID 1656 SNOMED CT ID 111196000

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 11 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Betamethasone	DMAHJEF	Approved	Small molecular drug	[1]
Cortisone Acetate	DMG8K57	Approved	Small molecular drug	[2]
Dapsone	DM4LT8A	Approved	Small molecular drug	[3]
Dexamethasone	DMMWZET	Approved	Small molecular drug	[4]
Hydrocortisone	DMGEMB7	Approved	Small molecular drug	[5]
Methylprednisolone	DM4BDON	Approved	Small molecular drug	[6]
Prednisolone	DMQ8FR2	Approved	Small molecular drug	[7]
Prednisone	DM2HG4X	Approved	Small molecular drug	[8]
Sulfapyridine	DMIUYFH	Approved	Small molecular drug	[9]
Sulfoxone	DMAYX6P	Approved	Small molecular drug	[9]
Triamcinolone	DM98IXF	Approved	Small molecular drug	[10]
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⏷ Show the Full List of 11 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 4 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
DSG3	TTEO4P8	Strong	Biomarker	[11]
FCAR	TTGUJAO	Definitive	Biomarker	[12]
TGM1	TT7A949	Definitive	Biomarker	[13]
TGM2	TT2F4OL	Definitive	Biomarker	[14]
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This Disease Is Related to 1 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
TGM3	DEOEB3Q	Disputed	Biomarker	[15]
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This Disease Is Related to 2 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
AGA	OTNWT1WB	Definitive	Biomarker	[16]
MYO9B	OTQ94R5K	Definitive	Biomarker	[17]
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References

1	Betamethasone FDA Label
2	Cortisone acetate FDA Label
3	Dapsone FDA Label
4	Dexamethasone FDA Label
5	Hydrocortisone FDA Label
6	Methylprednisolone FDA Label
7	Prednisolone FDA Label
8	Prednisone FDA Label
9	Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
10	Triamcinolone FDA Label
11	Diagnosis of autoimmune bullous diseases.J Dtsch Dermatol Ges. 2018 Sep;16(9):1077-1091. doi: 10.1111/ddg.13637.
12	Immunoexpression of IgA receptors (CD89, CD71) in dermatitis herpetiformis.Folia Histochem Cytobiol. 2017;55(4):212-220. doi: 10.5603/FHC.a2017.0024. Epub 2018 Feb 8.
13	Transglutaminase autoantibodies in dermatitis herpetiformis and celiac sprue.J Invest Dermatol. 2008 Feb;128(2):332-5. doi: 10.1038/sj.jid.5701041. Epub 2007 Aug 30.
14	Fibrillar-type dermatitis herpetiformis.Eur J Dermatol. 2019 Apr 1;29(2):115-120. doi: 10.1684/ejd.2019.3533.
15	Ex vivo Culture of Duodenal Biopsies from Patients with Dermatitis Herpetiformis Indicates that Transglutaminase 3 Antibody Production Occurs in the Gut.Acta Derm Venereol. 2018 Mar 13;98(3):366-372. doi: 10.2340/00015555-2849.
16	Coeliac disease.Paediatr Int Child Health. 2019 Feb;39(1):23-31. doi: 10.1080/20469047.2018.1504431. Epub 2018 Aug 13.
17	Myosin IXB gene region and gluten intolerance: linkage to coeliac disease and a putative dermatitis herpetiformis association.J Med Genet. 2008 Apr;45(4):222-7. doi: 10.1136/jmg.2007.053991. Epub 2007 Dec 12.