Details of Disease

General Information of Disease (ID: DISOJLKV)

• Disease Name: Bullous pemphigoid
• Synonyms: senile dermatitis herpetiformis; benign pemphigus; Parapemphigus; Old Age pemphigus; bullous pemphigoid
• Disease Class: EB41: Pemphigoid
• Definition: Bullous pemphigoid (BP) is the most common form of autoimmune bullous dermatosis.
• Disease Hierarchy:
  DIS3AYL6: Autoimmune bullous skin disease
  DISOJLKV: Bullous pemphigoid
• ICD Code:
  ICD-11: ICD-11: EB41.0
  ICD-10: ICD-10: L12.0
  Expand ICD-11: 'EB41.0
  Expand ICD-10: 'L12.0
• Disease Identifiers:
  MONDO ID: MONDO_0019082
  MESH ID: D010391
  UMLS CUI: C0030805
  MedGen ID: 10620
  Orphanet ID: 703
  SNOMED CT ID: 77090002

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 3 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Nomacopan	DML6E6G	Phase 3	Recombinant protein	[1]
Bertilimumab	DMRC0EQ	Phase 2	Monoclonal antibody	[2]
TNT009	DMSKF29	Phase 1	NA	[3]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 8 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
IL31	TT1RJXK	Limited	Biomarker	[4]
HLA-DRB1	TTUXSTW	moderate	Biomarker	[5]
IL23A	TTC1GLB	moderate	Biomarker	[6]
DPP7	TTOYT5L	Strong	Biomarker	[7]
DPP9	TTNDUL7	Strong	Biomarker	[7]
DSG3	TTEO4P8	Strong	Biomarker	[8]
IL5	TTPREZD	Strong	Biomarker	[9]
SELL	TT2IYXF	Strong	Altered Expression	[10]

This Disease Is Related to 17 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CCL18	OT7JYSK9	moderate	Biomarker	[11]
DST	OTHZBM4X	moderate	Biomarker	[12]
HLA-DQB1	OTVVI3UI	moderate	Genetic Variation	[13]
C5AR2	OTP1Q82J	Strong	Biomarker	[14]
CCL26	OT2B7HR9	Strong	Altered Expression	[15]
DOCK3	OTF3YS2W	Strong	Biomarker	[16]
DSG1	OT11HC3A	Strong	Biomarker	[17]
DSP	OTB2MOP8	Strong	Biomarker	[18]
DSPP	OT1TYNDN	Strong	Genetic Variation	[19]
HSD17B6	OTSB55D2	Strong	Genetic Variation	[20]
IL17RC	OTEFOBSS	Strong	Altered Expression	[21]
LAD1	OT6YGTVX	Strong	Biomarker	[22]
MED1	OTOO24C4	Strong	Biomarker	[16]
PLEC	OTU4XDEG	Strong	Biomarker	[23]
PRPF6	OT3U0ABN	Strong	Biomarker	[24]
RNASE2	OT8Z4FNE	Strong	Biomarker	[25]
RNASE3	OTVE2XD1	Strong	Biomarker	[25]

References

• [1] ClinicalTrials.gov (NCT05061771) A Randomized, Part A Partial Blinded and Part B Double Blinded, Placebo-controlled 24-week Clinical Study to Evaluate the Efficacy and Safety of Nomacopan Therapy in Adult Patients With Bullous Pemphigoid Receiving Adjunct Oral Corticosteroid Therapy (ARREST-BP). U.S.National Institutes of Health.
• [2] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [3] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [4] Role of the Pruritic Cytokine IL-31 in Autoimmune Skin Diseases.Front Immunol. 2019 Jun 21;10:1383. doi: 10.3389/fimmu.2019.01383. eCollection 2019.
• [5] Association of HLA class I and class II alleles with bullous pemphigoid in Chinese Hans.J Dermatol Sci. 2018 Mar;89(3):258-262. doi: 10.1016/j.jdermsci.2017.11.014. Epub 2017 Dec 2.
• [6] NET Formation in Bullous Pemphigoid Patients With Relapse Is Modulated by IL-17 and IL-23 Interplay.Front Immunol. 2019 Apr 4;10:701. doi: 10.3389/fimmu.2019.00701. eCollection 2019.
• [7] Bullous pemphigoid induced by dipeptidyl peptidase-4 (DPP-4) inhibitors: a pharmacovigilance-pharmacodynamic/pharmacokinetic assessment through an analysis of the vigibase.Expert Opin Drug Saf. 2019 Nov;18(11):1099-1108. doi: 10.1080/14740338.2019.1668373. Epub 2019 Sep 26.
• [8] Validation of chemiluminescent enzyme immunoassay in detection of autoantibodies in pemphigus and pemphigoid.J Dermatol Sci. 2017 Mar;85(3):208-215. doi: 10.1016/j.jdermsci.2016.12.007. Epub 2016 Dec 6.
• [9] The Role of Eosinophils in Bullous Pemphigoid: A Developing Model of Eosinophil Pathogenicity in Mucocutaneous Disease.Front Med (Lausanne). 2018 Jul 10;5:201. doi: 10.3389/fmed.2018.00201. eCollection 2018.
• [10] Expression of selected integrins and selectins in bullous pemphigoid.Mediators Inflamm. 2007;2007:31051. doi: 10.1155/2007/31051. Epub 2007 Apr 2.
• [11] Minocycline decreases Th2 chemokines from M2 macrophages: Possible mechanisms for the suppression of bullous pemphigoid by traditional bullous disease drugs.Exp Dermatol. 2018 Nov;27(11):1268-1272. doi: 10.1111/exd.13779. Epub 2018 Oct 9.
• [12] Development of bullous pemphigoid in junctional epidermolysis bullosa.J Eur Acad Dermatol Venereol. 2020 Mar;34(3):e146-e148. doi: 10.1111/jdv.16057. Epub 2020 Feb 13.
• [13] Gliptin-associated Bullous Pemphigoid and the Expression of Dipeptidyl Peptidase-4/CD26 in Bullous Pemphigoid.Acta Derm Venereol. 2019 May 1;99(6):602-609. doi: 10.2340/00015555-3166.
• [14] Tissue Destruction in Bullous Pemphigoid Can Be Complement Independent and May Be Mitigated by C5aR2.Front Immunol. 2018 Mar 15;9:488. doi: 10.3389/fimmu.2018.00488. eCollection 2018.
• [15] Increased Activity and Apoptosis of Eosinophils in Blister Fluids, Skin and Peripheral Blood of Patients with Bullous Pemphigoid.Acta Derm Venereol. 2017 Apr 6;97(4):464-471. doi: 10.2340/00015555-2581.
• [16] Non-bullous lesions as the first manifestation of bullous pemphigoid: A retrospective analysis of 181 cases.J Dermatol. 2017 Jul;44(7):742-746. doi: 10.1111/1346-8138.13782. Epub 2017 Mar 3.
• [17] Accuracy of molecular diagnostics in pemphigus and bullous pemphigoid: comparison of commercial and modified mosaic indirect immunofluorescence tests as well as enzyme-linked immunosorbent assays.Postepy Dermatol Alergol. 2017 Feb;34(1):21-27. doi: 10.5114/ada.2017.65617. Epub 2017 Feb 7.
• [18] The molecular genetics of basement membrane diseases.Arch Dermatol. 1993 Dec;129(12):1557-65.
• [19] Bullous pemphigoid associated with dipeptidyl peptidase-4 inhibitors. A case series and analysis of cases reported in the Spanish pharmacovigilance database.Int J Dermatol. 2020 Feb;59(2):197-206. doi: 10.1111/ijd.14658. Epub 2019 Oct 12.
• [20] Polymorphisms in the Mitochondrial Genome Are Associated With Bullous Pemphigoid in Germans.Front Immunol. 2019 Nov 22;10:2200. doi: 10.3389/fimmu.2019.02200. eCollection 2019.
• [21] Characteristic Pattern of IL-17RA, IL-17RB, and IL-17RC in Monocytes/Macrophages and Mast Cells From Patients With Bullous Pemphigoid.Front Immunol. 2019 Sep 11;10:2107. doi: 10.3389/fimmu.2019.02107. eCollection 2019.
• [22] Checkpoint Inhibition May Trigger the Rare Variant of Anti-LAD-1 IgG-Positive, Anti-BP180 NC16A IgG-Negative Bullous Pemphigoid.Front Immunol. 2019 Aug 14;10:1934. doi: 10.3389/fimmu.2019.01934. eCollection 2019.
• [23] Structural proteins of the dermal-epidermal junction targeted by autoantibodies in pemphigoid diseases.Exp Dermatol. 2017 Dec;26(12):1154-1162. doi: 10.1111/exd.13446.
• [24] Characterization of bullous pemphigoid antibodies by use of recombinant bullous pemphigoid antigen proteins.J Invest Dermatol. 1991 Oct;97(4):725-8. doi: 10.1111/1523-1747.ep12484223.
• [25] Mechanisms of pathogenic effects of eosinophil cationic protein and eosinophil-derived neurotoxin on human keratinocytes.Exp Dermatol. 2018 Dec;27(12):1322-1327. doi: 10.1111/exd.13782. Epub 2018 Oct 22.