MCID: BLL006
MIFTS: 61

Bullous Pemphigoid

Categories: Immune diseases, Rare diseases, Skin diseases
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Aliases & Classifications for Bullous Pemphigoid

MalaCards integrated aliases for Bullous Pemphigoid:

Name: Bullous Pemphigoid 11 19 58 75 14 16 71 31 33
Senile Dermatitis Herpetiformis 19 71
Benign Pemphigus 19 71
Pemphigoid, Bullous 43
Pemphigoid Bullous 53
Old Age Pemphigus 19
Parapemphigus 19
Pemphigoid 19

Characteristics:


Prevelance:

1-9/100000 (France, Germany, United Kingdom, Switzerland) 1-5/10000 (Europe, Germany) 58

Age Of Onset:

All ages 58

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 11 DOID:8506
ICD9CM 34 694.5
MeSH 43 D010391
NCIt 49 C84389
SNOMED-CT 68 77090002
ICD10 31 L12.0
MESH via Orphanet 44 D010391
ICD10 via Orphanet 32 L12.0
UMLS via Orphanet 72 C0030805
Orphanet 58 ORPHA703
ICD11 33 233308710
UMLS 71 C0030805 C0263311 C0392436

Summaries for Bullous Pemphigoid

GARD: 19 Bullous pemphigoid is a skin disorder characterized by large blisters. The blisters are usually located on the arms, legs, or middle of the body. In some people, the mouth or genitals are also affected. The blisters may break open and form ulcers or open sores. Bullous pemphigoid usually occurs in older persons and is rare in young people. Symptoms may come and go. Bullous pemphigoid is an autoimmune disorder which occurs when the body's immune system attacks and destroys healthy body tissue by mistake.

MalaCards based summary: Bullous Pemphigoid, also known as senile dermatitis herpetiformis, is related to epidermolysis bullosa acquisita and dermatitis herpetiformis. An important gene associated with Bullous Pemphigoid is HLA-DQB1 (Major Histocompatibility Complex, Class II, DQ Beta 1), and among its related pathways/superpathways are Signal Transduction and ERK Signaling. The drugs Hydrocortisone succinate and Hydrocortisone acetate have been mentioned in the context of this disorder. Affiliated tissues include skin, salivary gland and bone, and related phenotypes are diabetes mellitus and autoimmunity

Orphanet: 58 A rare autoimmune bullous skin disease characterized by acquired, subepidermal tense bullae occurring on normal of inflamed skin and that is typically widespread (occurring in the flexor regions of the proximal arms and legs, in the armpits, groin and the abdomen) and often associated with pruritus. The evolution is typically chronic with spontaneous exacerbations and remission.

Disease Ontology: 11 A pemphigoid that is characterized by large blisters.

Wikipedia: 75 Bullous pemphigoid (type of pemphigoid) is an autoimmune pruritic skin disease which typically occurs in... more...

Related Diseases for Bullous Pemphigoid

Diseases related to Bullous Pemphigoid via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 803)
# Related Disease Score Top Affiliating Genes
1 epidermolysis bullosa acquisita 31.9 LAMC2 LAMA3 LAD1 ITGB4 HLA-DRB1 DST
2 dermatitis herpetiformis 31.6 IL5 HLA-DRB1 HLA-DQB1 CCL11
3 hypereosinophilic syndrome 31.6 RNASE3 IL5 CCL17 CCL11
4 pemphigoid gestationis 31.5 RNASE3 PPL HLA-DRB1 EVPL DST DSG3
5 dermatitis 31.5 RNASE3 IL5 DSG1 CCL17 CCL11
6 scabies 31.4 IL5 DST COL17A1
7 autoimmune disease 31.4 IL5 HLA-DRB1 HLA-DQB1 DSG3 DSG1 COL17A1
8 erythema multiforme 31.2 IL5 DSP CCL17
9 pseudomembranous conjunctivitis 31.2 PPL EVPL DST DSG3 DSG1 COL17A1
10 epidermolysis bullosa, junctional 4, intermediate 31.1 LAMB3 LAD1 DST COL17A1
11 dermatitis, atopic 31.1 RNASE3 IL5 CCL17 CCL11
12 contact dermatitis 31.1 RNASE3 IL5 CCL17 CCL11
13 pemphigus 31.1 PPL HLA-DRB1 HLA-DQB1 EVPL DST DSP
14 lichen planus 31.0 LAMC2 DST DSG3 DSG1 COL17A1
15 pompholyx 31.0 DST COL17A1
16 epidermolysis bullosa, junctional 1a, intermediate 30.9 LAMC2 LAMB3 LAMA3 ITGB4 COL17A1
17 alopecia 30.9 HLA-DRB1 DSP COL17A1
18 intermediate generalized junctional epidermolysis bullosa 30.8 LAMC2 LAMB3 LAMA3 ITGB4 COL17A1
19 basal cell carcinoma 30.8 LAMC2 ITGB4 ITGA6 DST COL17A1
20 pemphigus vulgaris, familial 30.8 PPL HLA-DRB1 EVPL DST DSP DSG3
21 benign chronic pemphigus 30.8 DSP DSG3 DSG1
22 allergic disease 30.8 RNASE3 IL5 CCL17 CCL11
23 pemphigoid 30.8 RNASE3 PPL PLEC LAMB3 LAMA3 ITGB4
24 epidermolysis bullosa 30.7 PLEC LAMC2 LAMB3 LAMA3 LAD1 ITGB4
25 ocular cicatricial pemphigoid 30.7 ITGB4 ITGA6
26 pemphigus foliaceus 30.7 PPL HLA-DRB1 EVPL DST DSP DSG3
27 chronic urticaria 30.7 RNASE3 IL5 HLA-DRB1 HLA-DQB1
28 chronic conjunctivitis 30.7 RNASE3 IL5 CCL11
29 esophagitis 30.7 RNASE3 IL5 DSG1 CCL11
30 conjunctival disease 30.7 RNASE3 IL5 CCL11
31 cicatricial pemphigoid 30.7 LAMC2 LAMA3 LAD1 ITGB4 ITGA6 HLA-DQB1
32 herpetiform pemphigus 30.6 DSG3 DSG1
33 keratoconjunctivitis 30.6 RNASE3 IL5 CCL11
34 allergic contact dermatitis 30.6 RNASE3 IL5 CCL17 CCL11
35 allergic asthma 30.6 RNASE3 IL5 CCL17 CCL11
36 common cold 30.6 RNASE3 IL5 CCL11
37 proteasome-associated autoinflammatory syndrome 1 30.5 RNASE3 IL5 CCL11
38 aspergillosis 30.5 IL5 HLA-DRB1 CCL17
39 junctional epidermolysis bullosa non-herlitz type 30.5 PLEC LAMC2 LAMB3 LAMA3 ITGB4 DST
40 mite infestation 30.5 IL5 DST COL17A1
41 silicosis 30.4 HLA-DRB1 HLA-DQB1 DSG1
42 conjunctivitis 30.4 RNASE3 IL5 CCL11
43 pemphigus erythematosus 30.4 DSP DSG3
44 chronic eosinophilic pneumonia 30.4 RNASE3 IL5 CCL11
45 respiratory failure 30.4 RNASE3 IL5 CCL17 CCL11
46 eosinophilic gastroenteritis 30.4 RNASE3 IL5 CCL11
47 gastroenteritis 30.4 RNASE3 IL5 CCL11
48 parasitic helminthiasis infectious disease 30.3 RNASE3 IL5 CCL11
49 rubella 30.3 IL5 HLA-DRB1 HLA-DQB1
50 bullous impetigo 30.3 DSG3 DSG1

Comorbidity relations with Bullous Pemphigoid via Phenotypic Disease Network (PDN):


Acute Cystitis Decubitus Ulcer
Deficiency Anemia Heart Disease
Pemphigus Protein-Energy Malnutrition
Schizophreniform Disorder

Graphical network of the top 20 diseases related to Bullous Pemphigoid:



Diseases related to Bullous Pemphigoid

Symptoms & Phenotypes for Bullous Pemphigoid

Human phenotypes related to Bullous Pemphigoid:

58 30 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 diabetes mellitus 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000819
2 autoimmunity 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002960
3 erythema 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0010783
4 macule 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0012733
5 weight loss 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001824
6 eczema 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000964
7 urticaria 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001025
8 abnormal blistering of the skin 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0008066
9 recurrent infections 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002719
10 psoriasiform dermatitis 58 30 Frequent (33%) Frequent (79-30%)
HP:0003765

GenomeRNAi Phenotypes related to Bullous Pemphigoid according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.14 CCL11 CCL17 COL17A1 DSG1 DSG3 DSP
2 no effect GR00402-S-2 10.14 CCL11 COL17A1 DSG1 DSG3 DST HLA-DRB1

MGI Mouse Phenotypes related to Bullous Pemphigoid:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.21 CCL17 COL17A1 DSG3 DSP DST HLA-DQB1
2 digestive/alimentary MP:0005381 10.02 DSG3 DSP DST HLA-DQB1 IL5 ITGA6
3 renal/urinary system MP:0005367 10.01 COL17A1 DST HLA-DQB1 ITGA6 ITGB4 LAMB3
4 immune system MP:0005387 10 CCL11 CCL17 COL17A1 DSG3 DSP DST
5 craniofacial MP:0005382 9.86 DSG3 DSP DST ITGA6 ITGB4 LAMA3
6 respiratory system MP:0005388 9.61 CCL11 DSG3 HLA-DQB1 IL5 ITGA6 ITGB4
7 integument MP:0010771 9.44 COL17A1 DSG1 DSG3 DSP DST EVPL

Drugs & Therapeutics for Bullous Pemphigoid

Drugs for Bullous Pemphigoid (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 104)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Hydrocortisone succinate Approved Phase 4 2203-97-6 3643
2
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
3
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 3640 5754
4
Clobetasol Approved, Experimental, Investigational Phase 4 25122-46-7, 25122-41-2 2791 5311051 32798
5
Cholecalciferol Approved, Nutraceutical, Vet_approved Phase 4 67-97-0, 1406-16-2 5280795 10883523
6 Calciferol Phase 4
7 Calcium, Dietary Phase 4
8 Hydrocortisone 17-butyrate 21-propionate Phase 4
9 Immunoglobulins, Intravenous Phase 4
10 Immunoglobulin E Phase 4
11
Calcium Nutraceutical Phase 4 7440-70-2 271
12
Benralizumab Approved, Investigational Phase 3 1044511-01-4
13
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
14
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
15
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
16
Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
17
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 4894 5755
18
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5 1875
19
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 4159 6741
20
Mycophenolic acid Approved, Investigational Phase 2, Phase 3 24280-93-1 446541
21
Levoleucovorin Approved, Experimental, Investigational Phase 2, Phase 3 68538-85-2, 58-05-9, 73951-54-9 149436 6006
22
Triamcinolone Approved, Vet_approved Phase 2, Phase 3 124-94-7 31307
23
Dapsone Approved, Investigational Phase 2, Phase 3 80-08-0 2955
24
Omalizumab Approved, Investigational Phase 3 242138-07-4
25
Rituximab Approved Phase 3 174722-31-7
26
Nicotinamide Approved, Investigational Phase 3 98-92-0 936
27
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
28
Folic acid Approved, Nutraceutical, Vet_approved Phase 2, Phase 3 59-30-3 6037
29
Niacin Approved, Investigational, Nutraceutical Phase 3 59-67-6 938
30
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7 4897
31
Cortisone Experimental Phase 2, Phase 3 53-06-5 222786
32 Immunoglobulins Phase 3
33 Antibodies Phase 3
34 gamma-Globulins Phase 3
35 Rho(D) Immune Globulin Phase 3
36 Gentamicins Phase 3
37 Olive Phase 3
38 Alkylating Agents Phase 3
39 Antineoplastic Agents, Alkylating Phase 3
40 Antineoplastic Agents, Hormonal Phase 2, Phase 3
41 Hormones Phase 2, Phase 3
42 Hormone Antagonists Phase 2, Phase 3
43 glucocorticoids Phase 2, Phase 3
44 Anti-Inflammatory Agents Phase 2, Phase 3
45 Immunosuppressive Agents Phase 2, Phase 3
46 Anti-Bacterial Agents Phase 2, Phase 3
47 Anti-Infective Agents Phase 2, Phase 3
48 Antitubercular Agents Phase 2, Phase 3
49
Methylprednisolone Acetate Phase 2, Phase 3 584547
50 Antibiotics, Antitubercular Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 62)
# Name Status NCT ID Phase Drugs
1 Influence of Dermocorticoids on Bone Mineral Density in Patients With Bullous Pemphigoid Unknown status NCT03926377 Phase 4
2 Evaluation of Fluid Retention Due to Superpotent Topical Corticosteroid in Patients With Bullous Pemphigoid Unknown status NCT02360202 Phase 4 Clobetasol Propionate cream treatment
3 An Open Label Case Series on the Effects of Xolair (Omalizumab) in Bullous Pemphigoid Completed NCT00472030 Phase 4 Omalizumab;prednisone
4 Assessment of Rituximab Efficiency and Tolerance in Treatment of Bullous Pemphigoid. Completed NCT00525616 Phase 3 Mabthera
5 Comparison of Monotherapy With Protracted Superpotent Topical Steroids to Superpotent Topical Steroids Associated With Methotrexate in Bullous Pemphigoid Completed NCT02313870 Phase 3 clobetasol propionate + Methotrexate;clobetasol propionate alone
6 NPB-01(Intravenous Immunoglobulin) Therapy for Patients With Bullous Pemphigoid Unresponsive to Corticosteroids: Randomized, Double-Blind, Placebo Control,Parallel Assignment Study(Phase III) Completed NCT01408550 Phase 3 NPB-01;Placebo
7 Pharmacokinetics Study of Mycophenolic Acid in Patients With an Autoimmune Bullous Dermatose, Pemphigus or Cicatricial Pemphigoid. Completed NCT02993133 Phase 3 Cellcept® in autoimmune bullous dermatoses
8 Ozonated Olive Oil in Treatment of Pemphigus Vulgaris and Bullous Pemphigoid Compared to Conventional Topical Treatment Recruiting NCT05594472 Phase 3 Ozonated olive oil;Topical garamycin cream
9 A Multinational, Randomized, Double-blind, Parallel-group, Placebo-controlled Study to Investigate the Use of Benralizumab as a Treatment Option for Patients With Bullous Pemphigoid (FJORD) Recruiting NCT04612790 Phase 3
10 A Multicenter, Randomized, Double-Blind, Placebo-Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Dupilumab in Adult Patients With Bullous Pemphigoid Recruiting NCT04206553 Phase 2, Phase 3 dupilumab;Matching Placebo;Oral corticosteroids (OCS)
11 A Phase 2/3, Randomized, Double-Blinded, Placebo-Controlled, Parallel-Group Study to Investigate the Efficacy and Safety of Efgartigimod PH20 SC in Adult Participants With Bullous Pemphigoid Recruiting NCT05267600 Phase 2, Phase 3 Prednisone
12 Randomized Double Blind Double Dummy Control Trial Comparing the Safety and Efficacy of Rituximab Versus Oral Cyclophosphamide in Severe Forms of Mucous Membrane Pemphigoid Recruiting NCT03295383 Phase 3 Rituximab 1g IV;Cyclophosphamide 50Mg Oral Tablet;Placebo of Rituximab;Placebo Oral Tablet
13 Clinical Evaluation of New Treatment Strategy of Mucous Membrane Pemphigoid Using Large Dose of Prednisolone Plus Intra-lesional of Triamcinolone Acetonide Followed by Combination of Mycophenolate Mofetil, Dapsone and Low Dose Prednisolone Active, not recruiting NCT04744623 Phase 2, Phase 3 large dose of prednisolone plus intra-lesional of Triamcinolone Acetonide followed the systemic combination of mycophenolate mofetil (MMF), dapsone and low dose prednisolone in treatment of MMP
14 An Open-Label Study to Evaluate the Efficacy and Safety of Rituximab Combined With Omalizumab in Patients With Bullous Pemphigoid Not yet recruiting NCT04128176 Phase 3 Rituximab combined with Omalizumab
15 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) Withdrawn NCT05061771 Phase 3 nomacopan (rVA576)
16 Leflunomide Associated With Topical Corticosteroids for Bullous Pemphigoid. An Open Prospective Study Unknown status NCT00802243 Phase 2 leflunomide
17 Anti-IL-5 Therapy in Bullous Pemphigoid. Randomized, Placebo-controlled, Double-blind Study Evaluating the Effect of Anti-IL-5 Therapy in Patients With Bullous Pemphigoid. Completed NCT01705795 Phase 2 Mepolizumab (a-IL-5 antibody);Placebo
18 NPB-01(Intravenous Immunoglobulin) Therapy for Patients With Bullous Pemphigoid Unresponsive to Corticosteroids: Randomized, Double-Blind, Placebo Control,Parallel Assignment Study(PhaseⅡ). Completed NCT00809822 Phase 2 NPB-01;Placebo
19 Ixekizumab in the Treatment of Bullous Pemphigoid Completed NCT03099538 Phase 2 Ixekizumab
20 A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid Completed NCT00431119 Phase 2 Azathioprine or Mycophenolate mofetil
21 Rituximab in the Treatment of Patients With Bullous Pemphigoid Completed NCT00286325 Phase 1, Phase 2 Rituximab
22 A Phase IIa Open-label Single Arm Study of Safety and Efficacy of rVA576 in Adult Mild to Moderate Bullous Pemphigoid Subjects Completed NCT04035733 Phase 2 rVA576
23 Double-Blind, Randomized, Placebo-Controlled Trial of AKST4290 for Adjunctive Treatment of Mild to Moderate Bullous Pemphigoid Completed NCT04499235 Phase 2 Mometasone furoate;AKST4290;Placebo
24 An Open-Label, Proof of Concept Study Designed to Evaluate the Safety, Efficacy and Pharmacodynamic Effect of Bertilimumab in Patients With Newly Diagnosed, Moderate to Extensive Bullous Pemphigoid Completed NCT02226146 Phase 2
25 Phase I/II Clinical Trial Evaluating Rituximab in Ocular Cicatricial Pemphigoid Completed NCT00584935 Phase 1, Phase 2 Rituximab
26 The Use of Riboflavin/Ultraviolet A Cross-linked Human Donor Corneas as Carriers for the Boston Keratoprosthesis Completed NCT01582880 Phase 1, Phase 2 Riboflavin
27 Treatment of Bullous Pemphigoid With Avdoralimab (IPH5401), an Anti-C5aR1 Monoclonal Antibody Recruiting NCT04563923 Phase 2 Avdoralimab (IPH5401)
28 Efficacy and Safety of Ustekinumab in Bullous Pemphigoid Recruiting NCT04117932 Phase 2 Ustekinumab
29 Phase IIa Multicenter Clinical Trial to Determine the Feasibility and Safety of the Use of Adipose-derived Mesenchymal Stem Cells (ASC) in the Treatment of Patients With Cicatricial Conjunctivitis Associated With Lyell's Syndrome, Stevens-Johnson Syndrome and Pemphigoid of the Mucous Membranes With Ocular Involvement. Recruiting NCT05520086 Phase 1, Phase 2 Single Dose;Double Dose
30 Baricitinib for the Treatment of Ocular Mucous Membrane Pemphigoid Recruiting NCT05263505 Phase 2 Baricitinib 2 MG [Olumiant];Methotrexate;Azathioprine;Mycophenolate
31 Dexamethasone Solution and Dexamethasone in Mucolox™ for the Treatment of Oral Inflammatory Ulcerative Diseases Recruiting NCT04540133 Phase 2 dexamethasone 0.5mg/5ml solution;dexamethasone 0.5mg/5ml solution in Mucolox™
32 A Randomized, Double-blind, Placebo Controlled, Parallel Group Study Evaluating the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of QGE031 in the Treatment of Patients With Bullous Pemphigoid With Disease Refractory to Oral Steroid Treatment Terminated NCT01688882 Phase 2 QGE031;Placebo
33 A Randomized, Open-Label, Controlled Trial of Topical AC-203 in Subjects With Bullous Pemphigoid Terminated NCT03286582 Phase 2 AC-203 1% Topical Ointment;Clobetasol 0.05% Topical Ointment
34 A Phase 2, Multicentre, Single Arm, Pilot Study to Assess the Efficacy and the Safety of 150 mg Twice a Day Oral DF2156A in Patients With Active Bullous Pemphigoid. Terminated NCT01571895 Phase 2 DF2156A
35 Topical Infliximab in Autoimmune Eyes With Keratoprosthesis Withdrawn NCT02126020 Phase 1, Phase 2 topical infliximab
36 The Improvement of Limbal Epithelial Culture Technique for the Treatment of Unilateral Limbal Insufficiency by Using Collagenase to Isolate Limbal Stem Cells Unknown status NCT02202642 Phase 1
37 Safety, Tolerability and Activity of BIVV009 in Healthy Volunteers and Patients With Complement-mediated Disorders. A Single/Multiple Ascending Dose Phase 1 Study. Completed NCT02502903 Phase 1 BIV009
38 Clinical, Biological and Histological Characteristics of Bullous Pemphigoid Induced by antiPD-1/PDL-1 Therapy: A National Retrospective Cohort Study Unknown status NCT04641884
39 Dipeptidyl Peptidase-IV Inhibitors, Risk Factor for Development of Bullous Pemphigoid? : Multicenter Retrospective Study in France and Switzerland Unknown status NCT03636763 data report
40 The Association of Bullous Pemphigoid With Dipeptidyl-peptidase 4 Inhibitors: Α Ten-year Prospective Observational Study. Unknown status NCT04469582
41 Explore the Mechanisms of Pruritus in Bullous Pemphigoid Patients During Remission Unknown status NCT01265082
42 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Unknown status NCT02874079
43 Multicenter Prospective Observational Study Assessing Outcomes, Adverse Events, Treatment Patterns and Related Costs in Patients Diagnosed With Bullous Pemphigoid Unknown status NCT02837965 treatment with topical superpotent corticosteroid therapy;treatment with systemic therapy (methotrexate);treatment systemic therapy (prednisone);treatment with systemic therapy (prednisone);treatment with systemic therapy (doxycycline)
44 The Effects of Minor Salivary Gland Transplantation for Cicatrizing Conjunctivitis Unknown status NCT03839069
45 Study of the Periodontal Microbiota in Gingival Cicatricial Pemphigoid Patients. Bicentric Pilot Study on 30 Patients. Unknown status NCT04555681
46 An Open Randomized Comparative Trial of Four Different Dressings for Cutaneous Coverage in Pemphigus and Pemphigoid Unknown status NCT02365675
47 Evaluation of Cytokines and Immunoglobulins in Serum and Blister Fluids Appeared Before Treatment and Subsequently Under Treatment in Bullous Pemphigoid Completed NCT03856840
48 Clinical Characteristics of Pruritus and Evaluation of Quality of Life in Patients With Bullous Pemphigoid Completed NCT03272958
49 Impact of Neurological Diseases on the Prognosis of Bullous Pemphigoid: A Retrospective Study of 178 Patients Completed NCT03320798
50 Interest of Dosage of Anti-PB230, Anti-PB180 and Cytokines for Monitoring of Patients Suffering From Bullous Pemphigoid Completed NCT02883894

Search NIH Clinical Center for Bullous Pemphigoid

Inferred drug relations via UMLS 71 / NDF-RT 50 :


SULFAPYRIDINE PWDR

Cochrane evidence based reviews: pemphigoid, bullous

Genetic Tests for Bullous Pemphigoid

Anatomical Context for Bullous Pemphigoid

Organs/tissues related to Bullous Pemphigoid:

MalaCards : Skin, Salivary Gland, Bone, T Cells, Eye, Neutrophil, B Cells

Publications for Bullous Pemphigoid

Articles related to Bullous Pemphigoid:

(show top 50) (show all 7592)
# Title Authors PMID Year
1
C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin. 53 62
19863700 2010
2
Serum anti-BPAG1 auto-antibody is a novel marker for human melanoma. 53 62
20479946 2010
3
Sequential intramolecular epitope spreading of humoral responses to human BPAG2 in a transgenic model. 53 62
19812601 2010
4
Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid. 53 62
19751242 2010
5
Immunoglobulin A antibodies against desmoglein 1, envoplakin, periplakin and BP230 in a patient with atypical bullous pemphigoid. 53 62
20507390 2010
6
Plakins in striated muscle. 53 62
19768769 2010
7
Prevalence of autoantibodies to bullous pemphigoid antigens within the normal population. 53 62
20083697 2010
8
Anti-bullous pemphigoid 180 and 230 antibodies in a sample of unaffected subjects. 53 62
20083688 2010
9
Conjunctival interleukin-13 expression in mucous membrane pemphigoid and functional effects of interleukin-13 on conjunctival fibroblasts in vitro. 53 62
19910508 2009
10
Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. 53 62
19737550 2009
11
Basement membrane changes in lichen planopilaris. 53 62
19522713 2009
12
Experimental animal models of bullous pemphigoid. 53 62
19755945 2009
13
Plasmin plays a role in the in vitro generation of the linear IgA dermatosis antigen LADB97. 53 62
19158842 2009
14
Pemphigoid gestationis: clinical and laboratory evaluation. 53 62
19936176 2009
15
Cicatrising conjunctivitis with anti-basement membrane autoantibodies in ectodermal dysplasia. 53 62
18815422 2008
16
Calcineurin/NFAT-dependent regulation of 230-kDa bullous pemphigoid antigen (BPAG1) gene expression in normal human epidermal keratinocytes. 53 62
18353617 2008
17
Expression of vinculin in autoimmune cutaneous diseases. 53 62
18327013 2008
18
Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. 53 62
18289320 2008
19
Serum levels of IgE anti-BP180 and anti-BP230 autoantibodies in patients with bullous pemphigoid. 53 62
17920818 2008
20
Enzyme-linked immunosorbent assay in autoimmune blistering diseases: preliminary experience of the Dermatology Department of Cagliari. 53 62
18833046 2008
21
Role of BP180NC16a-enzyme-linked immunosorbent assay (ELISA) in the diagnosis of bullous pemphigoid in China. 53 62
18173596 2008
22
Experimental models for the autoimmune and inflammatory blistering disease, Bullous pemphigoid. 53 62
17879094 2007
23
Relative risk for cancer in mucous membrane pemphigoid associated with antibodies to the beta4 integrin subunit. 53 62
17524068 2007
24
A pathogenic role for IgE in autoimmunity: bullous pemphigoid IgE reproduces the early phase of lesion development in human skin grafted to nu/nu mice. 53 62
17611576 2007
25
[Comparison of urinary proteomics between steroid-sensitive and steroid-resistant minimal change nephrotic syndrome in children]. 53 62
17959526 2007
26
14-3-3 sigma isoform interacts with the cytoplasmic domain of the transmembrane BP180 in keratinocytes. 53 62
17443672 2007
27
Enzyme-linked immunosorbent assay using multimers of the 16th non-collagenous domain of the BP180 antigen for sensitive and specific detection of pemphigoid autoantibodies. 53 62
17697150 2007
28
Significance of IgG4 in the diagnosis of mucous membrane pemphigoid. 53 62
17344074 2007
29
Paraneoplastic pemphigus. 53 62
17683379 2007
30
Clinical and immunopathologic findings in oral lichen planus pemphigoides. 53 62
17531940 2007
31
[Paraneoplastic pemphigus]. 53 62
17513251 2007
32
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. 53 62
17263817 2007
33
Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases. 53 62
17222220 2007
34
Structural analysis of the plakin domain of bullous pemphigoid antigen1 (BPAG1) suggests that plakins are members of the spectrin superfamily. 53 62
17161423 2007
35
Oral pemphigoid autoantibodies preferentially target BP180 ectodomain. 53 62
17141573 2007
36
Relationship between cancer and oral pemphigoid patients with antibodies to alpha6-integrin. 53 62
17181734 2007
37
Antigen specificity in subsets of mucous membrane pemphigoid. 53 62
16810295 2006
38
Localisation of bullous pemphigoid antigen 180 (BP180) in cultured human keratinocytes: functionally relevant modification by calcium. 53 62
16906396 2006
39
Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum values. 53 62
17026501 2006
40
Autoimmune subepidermal blistering diseases in Uganda: correlation of autoantibody class with age of patients. 53 62
16961507 2006
41
Clinical and diagnostic features of mucous membrane pemphigoid. 53 62
17052040 2006
42
Thymus and activation regulated chemokine (TARC)/CCL17 and skin diseases. 53 62
16859899 2006
43
Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus vulgaris. 53 62
16882171 2006
44
Comparison of reactivity and epitope recognition between sera from American and Italian patients with oral pemphigoid. 53 62
16792670 2006
45
IgG4 as the predominant IgG subclass in pemphigoides gestationis. 53 62
16630180 2006
46
Epitopes in the linker subdomain region of envoplakin recognized by autoantibodies in paraneoplastic pemphigus patients. 53 62
16470171 2006
47
Interferon-gamma down-regulates expression of the 230-kDa bullous pemphigoid antigen gene (BPAG1) in epidermal keratinocytes via novel chimeric sequences of ISRE and GAS. 53 62
16512878 2006
48
Autoreactive T and B cells from bullous pemphigoid (BP) patients recognize epitopes clustered in distinct regions of BP180 and BP230. 53 62
16424234 2006
49
Identification of an epitope within human integrin alpha 6 subunit for the binding of autoantibody and its role in basement membrane separation in oral pemphigoid. 53 62
16424229 2006
50
Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity. 53 62
16403100 2006

Variations for Bullous Pemphigoid

Expression for Bullous Pemphigoid

Search GEO for disease gene expression data for Bullous Pemphigoid.

Pathways for Bullous Pemphigoid

Pathways related to Bullous Pemphigoid according to GeneCards Suite gene sharing:

(show all 27)
# Super pathways Score Top Affiliating Genes
1 13.67 LAMC2 LAMB3 LAMA3 IL5 DST DSP
2
Show member pathways
13.63 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 IL5
3
Show member pathways
13.27 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
4
Show member pathways
12.96 COL17A1 ITGA6 ITGB4 LAMA3 LAMB3 LAMC2
5
Show member pathways
12.83 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
6
Show member pathways
12.78 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
7
Show member pathways
12.63 COL17A1 DST ITGA6 ITGB4 LAMA3 LAMB3
8 12.57 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
9
Show member pathways
12.5 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
10
Show member pathways
12.47 PPL EVPL DSP DSG3 DSG1
11
Show member pathways
12.4 DSP DST EVPL PLEC PPL
12
Show member pathways
12.37 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
13
Show member pathways
12.32 LAMC2 LAMB3 LAMA3 ITGA6
14 12.1 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
15
Show member pathways
11.99 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
16
Show member pathways
11.91 DST DSP DSG1
17
Show member pathways
11.9 PLEC DSP DSG3 DSG1
18 11.87 PLEC ITGB4 ITGA6 DSP
19 11.76 IL5 HLA-DRB1 HLA-DQB1
20 11.72 LAMC2 LAMB3 LAMA3 ITGA6
21 11.57 IL5 DSP CCL11
22 11.55 LAMC2 LAMB3 LAMA3
23 11.53 IL5 CCL17 CCL11
24 11.48 ITGB4 EVPL DST
25 11.39 IL5 CCL17 CCL11
26 10.67 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
27 10.51 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 IL5

GO Terms for Bullous Pemphigoid

Cellular components related to Bullous Pemphigoid according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 10.18 PPL PLEC HLA-DRB1 EVPL DST DSP
2 cornified envelope GO:0001533 10.07 PPL EVPL DSP DSG3 DSG1
3 basement membrane GO:0005604 9.97 LAMC2 LAMB3 LAMA3 LAD1 ITGB4 DST
4 desmosome GO:0030057 9.96 DSG1 DSG3 DSP EVPL PPL
5 cellular anatomical entity GO:0110165 9.87 PPL PLEC LAMB3 LAMA3 EVPL DST
6 laminin-5 complex GO:0005610 9.73 LAMB3 LAMA3
7 anchoring junction GO:0070161 9.61 PPL PLEC ITGB4 EVPL DST DSP
8 hemidesmosome GO:0030056 9.32 PLEC LAMA3 ITGB4 DST COL17A1

Biological processes related to Bullous Pemphigoid according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 cell-cell adhesion GO:0098609 10.07 LAMA3 ITGA6 DSP DSG3 DSG1
2 cell adhesion GO:0007155 10.07 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 DST
3 wound healing GO:0042060 10.02 DSP DST EVPL PLEC PPL
4 integrin-mediated signaling pathway GO:0007229 10.01 LAMA3 ITGB4 ITGA6 DST
5 tissue development GO:0009888 9.93 LAMC2 LAMB3 LAMA3
6 keratinocyte differentiation GO:0030216 9.92 PLEC EVPL DSP
7 intermediate filament cytoskeleton organization GO:0045104 9.85 DSP DST EVPL PLEC PPL
8 cell motility GO:0048870 9.83 DST ITGB4 PLEC
9 epidermis development GO:0008544 9.8 LAMC2 LAMB3 LAMA3 HLA-DRB1 EVPL DSP
10 nail development GO:0035878 9.78 ITGB4 ITGA6
11 immunoglobulin production involved in immunoglobulin-mediated immune response GO:0002381 9.51 HLA-DRB1 HLA-DQB1
12 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.49 HLA-DRB1 HLA-DQB1
13 hemidesmosome assembly GO:0031581 9.32 PLEC LAMA3 ITGB4 DST COL17A1

Molecular functions related to Bullous Pemphigoid according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cadherin binding GO:0045296 10.07 PPL PLEC LAD1 ITGA6 EVPL
2 integrin binding GO:0005178 9.92 LAMA3 ITGB4 ITGA6 DST
3 extracellular matrix structural constituent GO:0005201 9.86 COL17A1 LAMA3 LAMB3 LAMC2
4 MHC class II receptor activity GO:0032395 9.58 HLA-DRB1 HLA-DQB1
5 structural constituent of cytoskeleton GO:0005200 9.56 PPL PLEC HLA-DRB1 DSP
6 structural molecule activity GO:0005198 9.53 PPL PLEC LAMB3 LAMA3 LAD1 EVPL
7 neuregulin binding GO:0038132 9.46 ITGB4 ITGA6

Sources for Bullous Pemphigoid

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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