MCID: BLL006
MIFTS: 61

Bullous Pemphigoid

Categories: Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Bullous Pemphigoid

MalaCards integrated aliases for Bullous Pemphigoid:

Name: Bullous Pemphigoid 12 73 20 58 15 17 70 32
Senile Dermatitis Herpetiformis 20 70
Benign Pemphigus 20 70
Pemphigoid, Bullous 44
Pemphigoid Bullous 54
Old Age Pemphigus 20
Parapemphigus 20
Pemphigoid 20

Characteristics:

Orphanet epidemiological data:

58
bullous pemphigoid
Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:8506
ICD9CM 34 694.5
MeSH 44 D010391
NCIt 50 C84389
SNOMED-CT 67 77090002
ICD10 32 L12.0
MESH via Orphanet 45 D010391
ICD10 via Orphanet 33 L12.0
UMLS via Orphanet 71 C0030805
Orphanet 58 ORPHA703
UMLS 70 C0030805 C0263311 C0392436

Summaries for Bullous Pemphigoid

GARD : 20 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. In most patients, the condition goes away after several years. Bullous pemphigoid is an autoimmune disorder which occurs when the body's immune system attacks and destroys healthy body tissue by mistake. Treatment may include corticosteroids taken by mouth or applied to the skin. Medicines that suppress the immune system may also be prescribed. For some, antibiotics in the tetracycline family are useful.

MalaCards based summary : Bullous Pemphigoid, also known as senile dermatitis herpetiformis, is related to pemphigoid and lichen planus pemphigoides. An important gene associated with Bullous Pemphigoid is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways/superpathways are ERK Signaling and Integrin Pathway. The drugs Hydrocortisone acetate and Hydrocortisone have been mentioned in the context of this disorder. Affiliated tissues include skin, t cells and neutrophil, and related phenotypes are diabetes mellitus and autoimmunity

Disease Ontology : 12 A pemphigoid that is characterized by large blisters.

Wikipedia : 73 Bullous pemphigoid is an autoimmune pruritic skin disease preferentially in older people, aged over 60,... 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 669)
# Related Disease Score Top Affiliating Genes
1 pemphigoid 32.7 ITGB4 ITGA6 IL5 HLA-DRB1 HLA-DQB1 DST
2 lichen planus pemphigoides 32.5 ITGB4 DST COL17A1
3 dermatitis herpetiformis 31.8 IL5 HLA-DQB1 CCL11
4 conjunctivitis 31.3 RNASE3 IL5 CCL11
5 autoimmune disease 31.3 IL5 HLA-DRB1 HLA-DQB1 DSG3 DSG1 COL17A1
6 linear iga disease 31.2 LAD1 ITGB4 DST COL17A1
7 dermatitis 31.2 RNASE3 IVL IL5 DSG1 CCL11
8 epidermolysis bullosa acquisita 31.1 LAMC2 LAMA3 LAD1 ITGB4 HLA-DRB1 DST
9 pemphigus 31.0 PPL HLA-DRB1 HLA-DQB1 EVPL DST DSP
10 pompholyx 30.9 DST COL17A1
11 pemphigoid gestationis 30.8 RNASE3 IL5 HLA-DRB1 DST DSG3 DSG1
12 pseudomembranous conjunctivitis 30.8 PPL LAMA3 EVPL DSP DSG3 DSG1
13 pemphigus foliaceus 30.8 PPL HLA-DRB1 EVPL DST DSG3 DSG1
14 cicatricial pemphigoid 30.7 LAMC2 LAMA3 LAD1 ITGB4 ITGA6 HLA-DRB1
15 conjunctival disease 30.7 RNASE3 IL5 CCL11
16 chronic conjunctivitis 30.7 RNASE3 IL5 CCL11
17 dermatitis, atopic 30.7 RNASE3 IVL IL5 CCL11
18 keratoconjunctivitis 30.7 RNASE3 IL5 CCL11
19 lichen planus 30.7 LAMC2 IVL DST DSG3 DSG1 COL17A1
20 herpetiform pemphigus 30.6 DST DSG3 DSG1
21 ecthyma 30.6 DST DSG1
22 discoid lupus erythematosus 30.6 IVL HLA-DRB1
23 severe cutaneous adverse reaction 30.6 PPL HLA-DRB1 HLA-DQB1
24 keratosis 30.6 IVL DSP DSG1
25 proteasome-associated autoinflammatory syndrome 1 30.6 RNASE3 IL5 CCL11
26 esophagitis 30.5 RNASE3 IL5 DSG1 CCL11
27 pemphigus vulgaris, familial 30.5 PPL IVL HLA-DRB1 EVPL DST DSP
28 atopic keratoconjunctivitis 30.5 RNASE3 IL5
29 pemphigus erythematosus 30.4 DSP DSG3
30 ocular cicatricial pemphigoid 30.4 RNASE3 ITGB4 ITGA6 HLA-DRB1 HLA-DQB1
31 chronic eosinophilic pneumonia 30.4 RNASE3 IL5 CCL11
32 eosinophilic pneumonia 30.4 RNASE3 IL5 CCL11
33 gastroenteritis 30.4 RNASE3 IL5 CCL11
34 rubella 30.4 IL5 HLA-DRB1 HLA-DQB1
35 benign chronic pemphigus 30.4 IVL DSP DSG3 DSG1
36 bullous impetigo 30.3 DSG3 DSG1
37 pemphigus vegetans 30.3 PPL DSG1
38 epidermolysis bullosa 30.3 PLEC LAMC2 LAMB3 LAMA3 LAD1 IVL
39 churg-strauss syndrome 30.3 RNASE3 IL5 HLA-DRB1 CCL11
40 bronchitis 30.3 RNASE3 IL5 CCL11
41 epidermolysis bullosa, junctional, non-herlitz type 30.2 PLEC LAMC2 LAMB3 LAMA3 LAD1 ITGB4
42 recessive dystrophic epidermolysis bullosa 30.2 LAMB3 IVL COL17A1
43 mite infestation 30.2 IL5 COL17A1
44 junctional epidermolysis bullosa 30.1 PLEC LAMC2 LAMB3 LAMA3 LAD1 ITGB4
45 subcorneal pustular dermatosis 29.9 PPL EVPL DST DSP DSG3 DSG1
46 paraneoplastic pemphigus 29.9 PPL HLA-DRB1 HLA-DQB1 EVPL DST DSP
47 bronchiolitis 29.9 RNASE3 IL5 CCL11
48 aplasia cutis congenita, nonsyndromic 29.9 PLEC ITGB4
49 pyloric atresia 29.9 PLEC LAMC2 LAD1 ITGB4 ITGA6 COL17A1
50 iga pemphigus 29.8 PPL EVPL 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 31 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 diabetes mellitus 58 31 hallmark (90%) Very frequent (99-80%) HP:0000819
2 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
3 erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0010783
4 macule 58 31 hallmark (90%) Very frequent (99-80%) HP:0012733
5 weight loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0001824
6 eczema 58 31 hallmark (90%) Very frequent (99-80%) HP:0000964
7 urticaria 58 31 hallmark (90%) Very frequent (99-80%) HP:0001025
8 abnormal blistering of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0008066
9 recurrent infections 58 31 hallmark (90%) Very frequent (99-80%) HP:0002719
10 psoriasiform dermatitis 58 31 frequent (33%) Frequent (79-30%) HP:0003765

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00249-S 9.55 COL17A1 IL5 ITGA6 IVL
2 Decreased viability GR00381-A-1 9.55 HLA-DQB1
3 Decreased viability GR00381-A-3 9.55 HLA-DQB1
4 Decreased viability GR00386-A-1 9.55 EVPL HLA-DRB1 ITGA6 IVL LAMB3
5 Decreased viability GR00402-S-2 9.55 DSP EVPL HLA-DQB1 ITGB4 LAMB3 LAMC2

MGI Mouse Phenotypes related to Bullous Pemphigoid:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.91 DSG3 DSP HLA-DQB1 IL5 ITGA6 ITGB4
2 immune system MP:0005387 9.9 CCL11 DSG3 DSP HLA-DQB1 IL5 ITGA6
3 craniofacial MP:0005382 9.87 DSG3 DSP ITGA6 ITGB4 LAMA3 LAMB3
4 integument MP:0010771 9.7 DSG1 DSG3 DSP EVPL ITGA6 ITGB4
5 respiratory system MP:0005388 9.28 CCL11 DSG3 HLA-DQB1 IL5 ITGA6 ITGB4

Drugs & Therapeutics for Bullous Pemphigoid

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

(show top 50) (show all 109)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
2
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754
3
Vitamin D Approved, Nutraceutical, Vet_approved Phase 4 1406-16-2
4 Hydrocortisone hemisuccinate Phase 4
5 Calciferol Phase 4
6 Hydrocortisone 17-butyrate 21-propionate Phase 4
7 Calcium, Dietary Phase 4
8 Immunoglobulins, Intravenous Phase 4
9 Immunoglobulin E Phase 4
10
Calcium Nutraceutical Phase 4 7440-70-2 271
11
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
12
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
13
Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
14
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
15
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
16
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
17
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
18
Mycophenolic acid Approved Phase 2, Phase 3 24280-93-1 446541
19
Prednisone Approved, Vet_approved Phase 2, Phase 3 53-03-2 5865
20
Levoleucovorin Approved, Investigational Phase 2, Phase 3 68538-85-2 149436
21
Triamcinolone Approved, Vet_approved Phase 2, Phase 3 124-94-7 31307
22
Dapsone Approved, Investigational Phase 2, Phase 3 80-08-0 2955
23
Omalizumab Approved, Investigational Phase 3 242138-07-4
24
rituximab Approved Phase 3 174722-31-7 10201696
25
Doxycycline Approved, Investigational, Vet_approved Phase 3 564-25-0 54671203
26
Benralizumab Approved, Investigational Phase 3 1044511-01-4
27
Nicotinamide Approved, Investigational Phase 3 98-92-0 936
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
31
Cortisone Experimental Phase 2, Phase 3 53-06-5 222786
32 gamma-Globulins Phase 3
33 Rho(D) Immune Globulin Phase 3
34 Alkylating Agents Phase 3
35 Hormone Antagonists Phase 2, Phase 3
36 glucocorticoids Phase 2, Phase 3
37 Hormones Phase 2, Phase 3
38 Anti-Inflammatory Agents Phase 2, Phase 3
39 Antibiotics, Antitubercular Phase 2, Phase 3
40 Immunosuppressive Agents Phase 2, Phase 3
41 Methylprednisolone Acetate Phase 2, Phase 3
42 Anti-Infective Agents Phase 2, Phase 3
43 Anti-Bacterial Agents Phase 2, Phase 3
44 Antitubercular Agents Phase 2, Phase 3
45 Antineoplastic Agents, Hormonal Phase 2, Phase 3
46 Vitamin B9 Phase 2, Phase 3
47 Folic Acid Antagonists Phase 2, Phase 3
48 Vitamin B Complex Phase 2, Phase 3
49 Folate Phase 2, Phase 3
50 Antiparasitic Agents Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 57)
# 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 Comparison of Monotherapy With Protracted Superpotent Topical Steroids to Superpotent Topical Steroids Associated With Methotrexate in Bullous Pemphigoid Unknown status NCT02313870 Phase 3 clobetasol propionate + Methotrexate;clobetasol propionate alone
5 Assessment of Rituximab Efficiency and Tolerance in Treatment of Bullous Pemphigoid. Completed NCT00525616 Phase 3 Mabthera
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 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)
9 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
10 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
11 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) Not yet recruiting NCT04612790 Phase 3
12 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
13 Leflunomide Associated With Topical Corticosteroids for Bullous Pemphigoid. An Open Prospective Study Unknown status NCT00802243 Phase 2 leflunomide
14 A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid Completed NCT00431119 Phase 2 Azathioprine or Mycophenolate mofetil
15 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
16 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
17 Ixekizumab in the Treatment of Bullous Pemphigoid Completed NCT03099538 Phase 2 Ixekizumab
18 Rituximab in the Treatment of Patients With Bullous Pemphigoid Completed NCT00286325 Phase 1, Phase 2 Rituximab
19 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
20 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
21 Phase I/II Clinical Trial Evaluating Rituximab in Ocular Cicatricial Pemphigoid Completed NCT00584935 Phase 1, Phase 2 Rituximab
22 The Use of Riboflavin/Ultraviolet A Cross-linked Human Donor Corneas as Carriers for the Boston Keratoprosthesis Completed NCT01582880 Phase 1, Phase 2 Riboflavin
23 Efficacy and Safety of Ustekinumab in Bullous Pemphigoid Recruiting NCT04117932 Phase 2 Ustekinumab
24 Double-Blind, Randomized, Placebo-Controlled Trial of AKST4290 for Adjunctive Treatment of Mild to Moderate Bullous Pemphigoid Recruiting NCT04499235 Phase 2 Mometasone furoate;AKST4290;Placebo
25 Treatment of Bullous Pemphigoid With Avdoralimab (IPH5401), an Anti-C5aR1 Monoclonal Antibody Recruiting NCT04563923 Phase 2 Avdoralimab (IPH5401)
26 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™
27 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
28 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
29 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
30 Topical Infliximab in Autoimmune Eyes With Keratoprosthesis Withdrawn NCT02126020 Phase 1, Phase 2 topical infliximab
31 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
32 Phase I Study of Vorinostat in Combination With Niacinamide, and Etoposide for the Treatment of Patients With Relapsed and Refractory Lymphoid Malignancies Completed NCT00691210 Phase 1 Vorinostat;Niacinamide;Etoposide
33 Safety, Tolerability and Activity of BIVV009 in Healthy Volunteers and Patients With Complement-mediated Disorders. A Single/Multiple Ascending Dose Phase 1 Study. Active, not recruiting NCT02502903 Phase 1 BIV009
34 Dipeptidyl Peptidase-IV Inhibitors, Risk Factor for Development of Bullous Pemphigoid? : Multicenter Retrospective Study in France and Switzerland Unknown status NCT03636763 data report
35 Explore the Mechanisms of Pruritus in Bullous Pemphigoid Patients During Remission Unknown status NCT01265082
36 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)
37 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Unknown status NCT02874079
38 An Open Randomized Comparative Trial of Four Different Dressings for Cutaneous Coverage in Pemphigus and Pemphigoid Unknown status NCT02365675
39 Serum Free Fatty Acids Desaturase Activity Indices in Sera of Patients on Long-term High-dose Corticosteroid Treatment Unknown status NCT02872584 Prednisone
40 Evaluation of Cytokines and Immunoglobulins in Serum and Blister Fluids Appeared Before Treatment and Subsequently Under Treatment in Bullous Pemphigoid Completed NCT03856840
41 Comparison of Two Therapeutic Strategies of Dermoval in Treatment of Bullous Pemphigus Completed NCT00213421 Dermoval
42 Interest of Dosage of Anti-PB230, Anti-PB180 and Cytokines for Monitoring of Patients Suffering From Bullous Pemphigoid Completed NCT02883894
43 Clinical Characteristics of Pruritus and Evaluation of Quality of Life in Patients With Bullous Pemphigoid Completed NCT03272958
44 Impact of Neurological Diseases on the Prognosis of Bullous Pemphigoid: A Retrospective Study of 178 Patients Completed NCT03320798
45 Pruritus and Pemphigoid in Nursing Home Patients Completed NCT02823067
46 Clinical, Biological and Histological Characteristics of Bullous Pemphigoid Induced by antiPD-1/PDL-1 Therapy: A National Retrospective Cohort Study Recruiting NCT04641884
47 The Effects of Minor Salivary Gland Transplantation for Cicatrizing Conjunctivitis Recruiting NCT03839069
48 Proteomic and Metabolomic Lacrimal Fingerprint in Diverse Pathologies of the Ocular Surface Recruiting NCT04198740
49 Prevalence and Clinical Severity of Autoimmune Blistering Diseases Recruiting NCT02753777
50 The Association of Bullous Pemphigoid With Dipeptidyl-peptidase 4 Inhibitors: Α Ten-year Prospective Observational Study. Active, not recruiting NCT04469582

Search NIH Clinical Center for Bullous Pemphigoid

Inferred drug relations via UMLS 70 / NDF-RT 51 :


SULFAPYRIDINE PWDR

Cochrane evidence based reviews: pemphigoid, bullous

Genetic Tests for Bullous Pemphigoid

Anatomical Context for Bullous Pemphigoid

MalaCards organs/tissues related to Bullous Pemphigoid:

40
Skin, T Cells, Neutrophil, B Cells, Thyroid, Bone, Colon

Publications for Bullous Pemphigoid

Articles related to Bullous Pemphigoid:

(show top 50) (show all 4468)
# Title Authors PMID Year
1
C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin. 61 54
19863700 2010
2
Serum anti-BPAG1 auto-antibody is a novel marker for human melanoma. 61 54
20479946 2010
3
Sequential intramolecular epitope spreading of humoral responses to human BPAG2 in a transgenic model. 54 61
19812601 2010
4
Immunoglobulin A antibodies against desmoglein 1, envoplakin, periplakin and BP230 in a patient with atypical bullous pemphigoid. 54 61
20507390 2010
5
Plakins in striated muscle. 61 54
19768769 2010
6
Prevalence of autoantibodies to bullous pemphigoid antigens within the normal population. 61 54
20083697 2010
7
Anti-bullous pemphigoid 180 and 230 antibodies in a sample of unaffected subjects. 54 61
20083688 2010
8
Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. 61 54
19737550 2009
9
Basement membrane changes in lichen planopilaris. 54 61
19522713 2009
10
Experimental animal models of bullous pemphigoid. 54 61
19755945 2009
11
Plasmin plays a role in the in vitro generation of the linear IgA dermatosis antigen LADB97. 54 61
19158842 2009
12
Pemphigoid gestationis: clinical and laboratory evaluation. 54 61
19936176 2009
13
Cicatrising conjunctivitis with anti-basement membrane autoantibodies in ectodermal dysplasia. 61 54
18815422 2008
14
Calcineurin/NFAT-dependent regulation of 230-kDa bullous pemphigoid antigen (BPAG1) gene expression in normal human epidermal keratinocytes. 61 54
18353617 2008
15
Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. 61 54
18289320 2008
16
Expression of vinculin in autoimmune cutaneous diseases. 54 61
18327013 2008
17
Serum levels of IgE anti-BP180 and anti-BP230 autoantibodies in patients with bullous pemphigoid. 61 54
17920818 2008
18
Enzyme-linked immunosorbent assay in autoimmune blistering diseases: preliminary experience of the Dermatology Department of Cagliari. 54 61
18833046 2008
19
Role of BP180NC16a-enzyme-linked immunosorbent assay (ELISA) in the diagnosis of bullous pemphigoid in China. 61 54
18173596 2008
20
Experimental models for the autoimmune and inflammatory blistering disease, Bullous pemphigoid. 54 61
17879094 2007
21
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. 61 54
17611576 2007
22
[Comparison of urinary proteomics between steroid-sensitive and steroid-resistant minimal change nephrotic syndrome in children]. 54 61
17959526 2007
23
14-3-3 sigma isoform interacts with the cytoplasmic domain of the transmembrane BP180 in keratinocytes. 61 54
17443672 2007
24
Enzyme-linked immunosorbent assay using multimers of the 16th non-collagenous domain of the BP180 antigen for sensitive and specific detection of pemphigoid autoantibodies. 61 54
17697150 2007
25
Paraneoplastic pemphigus. 54 61
17683379 2007
26
Clinical and immunopathologic findings in oral lichen planus pemphigoides. 61 54
17531940 2007
27
[Paraneoplastic pemphigus]. 54 61
17513251 2007
28
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. 54 61
17263817 2007
29
Structural analysis of the plakin domain of bullous pemphigoid antigen1 (BPAG1) suggests that plakins are members of the spectrin superfamily. 54 61
17161423 2007
30
Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases. 54 61
17222220 2007
31
Localisation of bullous pemphigoid antigen 180 (BP180) in cultured human keratinocytes: functionally relevant modification by calcium. 54 61
16906396 2006
32
Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum values. 54 61
17026501 2006
33
Clinical and diagnostic features of mucous membrane pemphigoid. 61 54
17052040 2006
34
Autoimmune subepidermal blistering diseases in Uganda: correlation of autoantibody class with age of patients. 61 54
16961507 2006
35
Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus vulgaris. 54 61
16882171 2006
36
Thymus and activation regulated chemokine (TARC)/CCL17 and skin diseases. 54 61
16859899 2006
37
IgG4 as the predominant IgG subclass in pemphigoides gestationis. 54 61
16630180 2006
38
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. 61 54
16512878 2006
39
Epitopes in the linker subdomain region of envoplakin recognized by autoantibodies in paraneoplastic pemphigus patients. 61 54
16470171 2006
40
Autoreactive T and B cells from bullous pemphigoid (BP) patients recognize epitopes clustered in distinct regions of BP180 and BP230. 54 61
16424234 2006
41
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. 54 61
16403100 2006
42
Collagen XVII/BP180: a collagenous transmembrane protein and component of the dermoepidermal anchoring complex. 54 61
16197389 2005
43
Mucous membrane pemphigoid: an update. 54 61
16175044 2005
44
A case of bullous pemphigoid associated with autoantibodies targeting antigenic sites other than the NC16a domain of BP180. 54 61
16045678 2005
45
Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. 61 54
16120143 2005
46
Autoantibodies to human alpha6 integrin in patients with bullous pemphigoid. 61 54
16126949 2005
47
Transcriptional regulation of the 230-kDa bullous pemphigoid antigen gene expression by interferon regulatory factor 1 and interferon regulatory factor 2 in normal human epidermal keratinocytes. 54 61
15560761 2004
48
Retinoic acid disintegrated desmosomes and hemidesmosomes in stratified oral keratinocytes. 54 61
15482329 2004
49
Profile of autoantibody to basement membrane zone proteins in patients with mucous membrane pemphigoid: long-term follow up and influence of therapy. 54 61
15308120 2004
50
Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. 61 54
15243525 2004

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 26)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.71 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 IL5
2
Show member pathways
13.3 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
3
Show member pathways
12.95 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
4
Show member pathways
12.79 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
5
Show member pathways
12.74 RNASE3 IL5 HLA-DRB1 HLA-DQB1 CCL11
6 12.73 LAMC2 LAMB3 LAMA3 ITGA6 IL5
7
Show member pathways
12.69 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
8
Show member pathways
12.49 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
9
Show member pathways
12.38 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
10
Show member pathways
12.34 PPL IVL EVPL DSP DSG3 DSG1
11
Show member pathways
12.21 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
12
Show member pathways
12.08 LAMC2 LAMB3 LAMA3 ITGA6 HLA-DRB1 HLA-DQB1
13
Show member pathways
12.02 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
14 11.93 PLEC ITGB4 ITGA6 DSP
15
Show member pathways
11.86 PLEC DSP DSG3 DSG1
16
Show member pathways
11.85 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
17 11.81 LAMC2 LAMB3 LAMA3
18
Show member pathways
11.8 PPL PLEC EVPL DST DSP
19 11.77 HLA-DRB1 HLA-DQB1 DSG1
20 11.77 ITGA6 IL5 HLA-DRB1 HLA-DQB1
21 11.72 LAMC2 LAMB3 LAMA3 ITGA6
22 11.54 IL5 DSP CCL11
23 11.52 LAMC2 LAMB3 LAMA3
24 11.26 LAMA3 IVL ITGB4
25 11.18 ITGB4 ITGA6 COL17A1
26 10.76 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 extracellular exosome GO:0070062 10.1 PPL PLEC LAMA3 LAD1 IVL ITGB4
2 cell junction GO:0030054 10.02 PPL PLEC ITGB4 EVPL DST DSP
3 intermediate filament GO:0005882 9.8 PPL PLEC HLA-DRB1 EVPL DST DSP
4 cell-cell junction GO:0005911 9.73 DSP DSG3 DSG1 COL17A1
5 hemidesmosome GO:0030056 9.56 PLEC ITGB4 DST COL17A1
6 desmosome GO:0030057 9.55 PPL EVPL DSP DSG3 DSG1
7 MHC class II protein complex GO:0042613 9.46 HLA-DRB1 HLA-DQB1
8 cornified envelope GO:0001533 9.43 PPL IVL EVPL DSP DSG3 DSG1
9 basement membrane GO:0005604 9.17 LAMC2 LAMB3 LAMA3 LAD1 ITGB4 DST

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

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.97 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 DST
2 extracellular matrix organization GO:0030198 9.91 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
3 cell-cell adhesion GO:0098609 9.85 LAMA3 ITGA6 DSP DSG3 DSG1
4 keratinization GO:0031424 9.85 PPL IVL EVPL DSP DSG3 DSG1
5 wound healing GO:0042060 9.8 PPL PLEC EVPL DST DSP
6 integrin-mediated signaling pathway GO:0007229 9.78 LAMA3 ITGB4 ITGA6 DST
7 cornification GO:0070268 9.73 PPL IVL EVPL DSP DSG3 DSG1
8 keratinocyte differentiation GO:0030216 9.72 IVL EVPL DSP
9 tissue development GO:0009888 9.7 LAMC2 LAMB3 LAMA3
10 peptide cross-linking GO:0018149 9.67 IVL EVPL DSP
11 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.58 HLA-DRB1 HLA-DQB1
12 skin morphogenesis GO:0043589 9.57 ITGB4 ITGA6
13 nail development GO:0035878 9.56 ITGB4 ITGA6
14 intermediate filament cytoskeleton organization GO:0045104 9.55 PPL PLEC EVPL DST DSP
15 humoral immune response mediated by circulating immunoglobulin GO:0002455 9.54 HLA-DRB1 HLA-DQB1
16 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 9.51 HLA-DRB1 HLA-DQB1
17 epidermis development GO:0008544 9.5 LAMC2 LAMB3 LAMA3 HLA-DRB1 EVPL DSP
18 hemidesmosome assembly GO:0031581 9.23 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6

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

# Name GO ID Score Top Affiliating Genes
1 cadherin binding GO:0045296 9.65 PPL PLEC LAD1 ITGA6 EVPL
2 extracellular matrix structural constituent GO:0005201 9.46 LAMC2 LAMB3 LAMA3 COL17A1
3 insulin-like growth factor I binding GO:0031994 9.4 ITGB4 ITGA6
4 MHC class II receptor activity GO:0032395 9.37 HLA-DRB1 HLA-DQB1
5 neuregulin binding GO:0038132 9.32 ITGB4 ITGA6
6 structural constituent of cytoskeleton GO:0005200 9.26 PPL PLEC HLA-DRB1 DSP
7 structural molecule activity GO:0005198 9.23 PPL PLEC LAMB3 LAMA3 LAD1 EVPL

Sources for Bullous Pemphigoid

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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