MCID: BLL006
MIFTS: 63

Bullous Pemphigoid

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

Aliases & Classifications for Bullous Pemphigoid

MalaCards integrated aliases for Bullous Pemphigoid:

Name: Bullous Pemphigoid 12 75 53 59 15 17 72 33
Pemphigoid 53 37 33
Senile Dermatitis Herpetiformis 53 72
Benign Pemphigus 53 72
Pemphigoid, Bullous 44
Pemphigoid Bullous 55
Old Age Pemphigus 53
Parapemphigus 53

Characteristics:

Orphanet epidemiological data:

59
bullous pemphigoid
Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:8506
KEGG 37 H01650
ICD9CM 35 694.5
MeSH 44 D010391
SNOMED-CT 68 77090002 86142006
ICD10 33 L12 L12.0 L12.9
MESH via Orphanet 45 D010391
ICD10 via Orphanet 34 L12.0
UMLS via Orphanet 73 C0030805
Orphanet 59 ORPHA703
UMLS 72 C0030805 C0263311 C0392436

Summaries for Bullous Pemphigoid

KEGG : 37
Pemphigoid diseases are group of autoimmune blistering disorders of the skin and mucous membranes characterized by autoantibodies directed against structural proteins of the dermal-epidermal junction that clinically can manifest with urticarial lesions, tense blisters, and erosions which may involve the mucous membranes. Diseases in this group include bullous pemphigoid (BP), dermatitis herpetiformis, mucous membrane pemphigois, linear IgA bullous dermatosis, herpes gestationis, and epidermolysis bullosa acquisita. BP is the most common acquired bullous autoimmune dermatosis disease. It is primarily a disease of the elderly with an equal incidence in men and women. The clinical characteristics are the formation of tense blisters and pruritic urticarial erythema. Histopathologically, subepidermal blister formation associated with eosinophil infiltration is commonly observed in BP. The main autoantigens targeted by BP autoantibodies are hemidesmosomal transmembrane collagen XVII (COL17, also known as BP180/BPAG2) and the intracytoplasmic plakin family protein BP230. Most of these antibodies belong to the immunoglobulin G class. Diagnosis of BP relies on the clinical and histological aspects, but the most reliable clues are given by direct and indirect immunofluorescence. Three types of drugs, with distinct mechanisms of action, are used to treat BP: anti-inflammatory drugs, drugs designed to reduce the production of pathogenic antibodies, and treatments that increase the elimination of pathogenic antibodies from the serum of patients, such as high-dose intravenous immunoglobulin (IVIG) therapy and therapeutic plasma exchange.

MalaCards based summary : Bullous Pemphigoid, also known as pemphigoid, is related to lichen planus pemphigoides and linear iga disease. 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 Omalizumab and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include skin, eye and t cells, and related phenotypes are diabetes mellitus and abnormal blistering of the skin

Disease Ontology : 12 An autoimmune disease of skin and connective tissue characterized by large blisters.

NIH Rare Diseases : 53 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.

Wikipedia : 75 Bullous pemphigoid is an autoimmune pruritic skin disease preferentially in elderly people, that may... 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 632)
# Related Disease Score Top Affiliating Genes
1 lichen planus pemphigoides 33.7 ITGB4 DST COL17A1
2 linear iga disease 31.7 ITGB4 DST COL17A1
3 lichen planus 31.7 DST DSG3 COL17A1
4 conjunctivitis 31.6 RNASE3 IL5 CCL11
5 epidermolysis bullosa acquisita 31.4 LAMA3 ITGB4 DST COL17A1
6 autoimmune disease 31.4 TNFSF13B HLA-DRB1 HLA-DQB1 DSG1 COL17A1
7 pemphigoid gestationis 31.4 IL5 HLA-DRB1 DST COL17A1 CCL11
8 ocular cicatricial pemphigoid 31.0 RNASE3 ITGB4 ITGA6 HLA-DRB1 HLA-DQB1
9 lichen sclerosus 31.0 HLA-DRB1 HLA-DQB1 COL17A1
10 conjunctival disease 31.0 RNASE3 IL5 CCL11
11 chronic conjunctivitis 31.0 RNASE3 IL5 CCL11
12 hypereosinophilic syndrome 30.8 RNASE3 IL5 CCL17 CCL11
13 proteasome-associated autoinflammatory syndrome 1 30.8 RNASE3 IL5 CCL11
14 herpetiform pemphigus 30.8 DST DSG3 DSG1
15 pemphigus erythematosus 30.7 DSP DSG3
16 dermatitis 30.7 IL5 DSG1 CCL17 CCL11
17 gastroenteritis 30.7 RNASE3 IL5 CCL11
18 blepharoconjunctivitis 30.7 RNASE3 CCL11
19 chronic eosinophilic pneumonia 30.7 RNASE3 IL5 CCL11
20 cicatricial pemphigoid 30.6 LAMC2 LAMA3 ITGB4 ITGA6 DST COL17A1
21 esophagitis 30.6 IL5 DSG1 CCL11
22 bronchitis 30.6 RNASE3 IL5 CCL11
23 epidermolysis bullosa simplex 30.5 PLEC ITGB4 DST COL17A1
24 pemphigus vegetans 30.4 PPL DSG1
25 atopic keratoconjunctivitis 30.4 RNASE3 IL5
26 bullous impetigo 30.4 DSG3 DSG1
27 pemphigus 30.4 PPL EVPL DST DSP DSG3 DSG1
28 dermatitis, atopic 30.3 RNASE3 IL5 CCL17 CCL11
29 bronchiolitis 30.2 RNASE3 IL5 CCL11
30 allergic hypersensitivity disease 30.1 RNASE3 IL5 CCL17 CCL11
31 pemphigus foliaceus 30.0 PPL HLA-DRB1 EVPL DST DSG3 DSG1
32 rhinitis 29.9 RNASE3 IL5 CCL11
33 allergic asthma 29.9 RNASE3 IL5 CCL17 CCL11
34 epidermolysis bullosa junctionalis with pyloric atresia 29.9 PLEC ITGB4 ITGA6 DST COL17A1
35 keratoconjunctivitis 29.9 RNASE3 IL5 CCL17 CCL11
36 churg-strauss syndrome 29.8 RNASE3 IL5 CCL17
37 pyloric atresia 29.8 PLEC LAMC2 ITGB4 ITGA6 COL17A1
38 junctional epidermolysis bullosa 29.7 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 DST
39 eosinophilic pneumonia 29.6 RNASE3 IL5 CCL17 CCL11
40 epidermolysis bullosa 29.4 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
41 epidermolysis bullosa, junctional, non-herlitz type 29.1 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
42 paraneoplastic pemphigus 28.6 PPL HLA-DRB1 HLA-DQB1 EVPL DST DSP
43 bullous skin disease 28.3 PPL LAMA3 IL5 EVPL DST DSP
44 subcorneal pustular dermatosis 28.3 PPL EVPL DST DSG3 DSG1 COL17A1
45 cellulitis 28.2 RNASE3 PPL LAMA3 IL5 DST DSP
46 vesiculobullous skin disease 28.1 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
47 skin disease 26.5 RNASE3 PLEC LAMC2 LAMB3 LAMA3 ITGB4
48 immunodysregulation, polyendocrinopathy, and enteropathy, x-linked 11.7
49 autoimmune disease, multisystem, infantile-onset, 2 11.4
50 recessive dystrophic epidermolysis bullosa-generalized other 11.4

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:

59 32 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 diabetes mellitus 59 32 hallmark (90%) Very frequent (99-80%) HP:0000819
2 abnormal blistering of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0008066
3 erythema 59 32 hallmark (90%) Very frequent (99-80%) HP:0010783
4 macule 59 32 hallmark (90%) Very frequent (99-80%) HP:0012733
5 weight loss 59 32 hallmark (90%) Very frequent (99-80%) HP:0001824
6 urticaria 59 32 hallmark (90%) Very frequent (99-80%) HP:0001025
7 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
8 recurrent infections 59 32 hallmark (90%) Very frequent (99-80%) HP:0002719
9 eczema 59 32 hallmark (90%) Very frequent (99-80%) HP:0000964
10 psoriasiform dermatitis 59 32 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 no effect GR00402-S-1 9.92 CCL11 CCL17 COL17A1 DSG1 DSG3 DSP

MGI Mouse Phenotypes related to Bullous Pemphigoid:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10 DSG1 DSG3 DSP HLA-DQB1 IL5 ITGA6
2 digestive/alimentary MP:0005381 9.97 DSG3 DSP HLA-DQB1 IL5 ITGA6 ITGB4
3 craniofacial MP:0005382 9.91 DSG3 DSP ITGA6 ITGB4 LAMA3 LAMB3
4 immune system MP:0005387 9.9 CCL11 CCL17 DSG3 HLA-DQB1 IL5 ITGA6
5 integument MP:0010771 9.65 DSG1 DSG3 DSP EVPL ITGA6 ITGB4
6 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 118)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Omalizumab Approved, Investigational Phase 4 242138-07-4
2
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
3
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
4
Clobetasol Approved, Experimental, Investigational Phase 4 25122-46-7, 25122-41-2 32798 5311051
5
Hydrocortisone Approved, Vet_approved Phase 4 50-23-7 5754
6
Hydrocortisone acetate Approved, Vet_approved Phase 4 50-03-3
7
Vitamin D Approved, Nutraceutical, Vet_approved Phase 4 1406-16-2
8
Calcium Approved, Nutraceutical Phase 4 7440-70-2 271
9 Immunologic Factors Phase 4
10 Pharmaceutical Solutions Phase 4
11 Immunosuppressive Agents Phase 4
12 Immunoglobulin E Phase 4
13 Anti-Allergic Agents Phase 4
14 Antineoplastic Agents, Hormonal Phase 4
15 Respiratory System Agents Phase 4
16 Anti-Asthmatic Agents Phase 4
17 Olive Phase 4
18 Ophthalmic Solutions Phase 4
19 Cyclosporins Phase 4
20 Calcineurin Inhibitors Phase 4
21 Hormones Phase 4
22 glucocorticoids Phase 4
23 Hormone Antagonists Phase 4
24 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4
25 Anti-Inflammatory Agents Phase 4
26 Vitamins Phase 4
27 Calcium, Dietary Phase 4
28 Hydrocortisone 17-butyrate 21-propionate Phase 4
29 Hydrocortisone hemisuccinate Phase 4
30 Calciferol Phase 4
31
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
32
leucovorin Approved Phase 3 58-05-9 143 6006
33
Mycophenolic acid Approved Phase 3 24280-93-1 446541
34
Naltrexone Approved, Investigational, Vet_approved Phase 3 16590-41-3 5360515
35
Ethanol Approved Phase 3 64-17-5 702
36
rituximab Approved Phase 3 174722-31-7 10201696
37
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
38 Antimetabolites, Antineoplastic Phase 3
39 Folic Acid Antagonists Phase 3
40 Nucleic Acid Synthesis Inhibitors Phase 3
41 Rho(D) Immune Globulin Phase 3
42 gamma-Globulins Phase 3
43 Immunoglobulins, Intravenous Phase 3
44 Anti-Bacterial Agents Phase 3
45 Anti-Infective Agents Phase 3
46 Antitubercular Agents Phase 3
47 Antibiotics, Antitubercular Phase 3
48 Narcotics Phase 3
49 Narcotic Antagonists Phase 3
50 Peripheral Nervous System Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 58)
# Name Status NCT ID Phase Drugs
1 Evaluation of Fluid Retention Due to Superpotent Topical Corticosteroid in Patients With Bullous Pemphigoid Unknown status NCT02360202 Phase 4 Clobetasol Propionate cream treatment
2 An Open Label Case Series on the Effects of Xolair (Omalizumab) in Bullous Pemphigoid Completed NCT00472030 Phase 4 Omalizumab;prednisone
3 Treatment of Dry Eye Using 0.03% Tacrolimus Eye Drops: Prospective Double-Blind Randomized Study Completed NCT01850979 Phase 4 Tacrolimus;Olive Oil
4 Influence of Dermocorticoids on Bone Mineral Density in Patients With Bullous Pemphigoid Not yet recruiting NCT03926377 Phase 4
5 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
6 Assessment of Rituximab Efficiency and Tolerance in Treatment of Bullous Pemphigoid. Completed NCT00525616 Phase 3 Mabthera
7 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
8 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
9 Autologous ex Vivo Conjunctival Epithelial Cell Expansion for Ocular Surface Completed NCT00346450 Phase 3
10 A Randomized Placebo-Controlled, Crossover-Design Study of the Effects of Low Dose Naltrexone on Quality of Life as Measured by the Multiple Sclerosis Quality of Life Inventory (MSQLI54) Completed NCT00501696 Phase 3 4.5 mg Naltrexone;Naltrexone
11 Randomized Double Blind Double Dummy Control Trial Comparing the Safety and Efficacy of Rituximab Versus Oral Cyclophosphamide in Severe Forms of Mucous Membrane Pemphigoid Not yet recruiting NCT03295383 Phase 3 Rituximab 1g IV;Cyclophosphamide 50Mg Oral Tablet;Placebo of Rituximab;Placebo Oral Tablet
12 Leflunomide Associated With Topical Corticosteroids for Bullous Pemphigoid. An Open Prospective Study Unknown status NCT00802243 Phase 2 leflunomide
13 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
14 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
15 Ixekizumab in the Treatment of Bullous Pemphigoid Completed NCT03099538 Phase 2 Ixekizumab
16 A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid Completed NCT00431119 Phase 2 Azathioprine or Mycophenolate mofetil
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 Phase I/II Clinical Trial Evaluating Rituximab in Ocular Cicatricial Pemphigoid Completed NCT00584935 Phase 1, Phase 2 Rituximab
19 Rituximab in the Treatment of Patients With Bullous Pemphigoid Completed NCT00286325 Phase 1, Phase 2 Rituximab
20 The Use of Riboflavin/Ultraviolet A Cross-linked Human Donor Corneas as Carriers for the Boston Keratoprosthesis Completed NCT01582880 Phase 1, Phase 2 Riboflavin
21 A Phase IIa Open-label Single Arm Study of Safety and Efficacy of rVA576 in Adult Mild to Moderate Bullous Pemphigoid Subjects Recruiting NCT04035733 Phase 2
22 A Randomized, Open-Label, Controlled Trial of Topical AC-203 in Subjects With Bullous Pemphigoid Recruiting NCT03286582 Phase 2 AC-203 1% Topical Ointment;Clobetasol 0.05% Topical Ointment
23 Translational Stem Cell Research in Ophthalmology - Regenerating the Anterior Cornea Through Standardized Transplantation of Limbal Epithelial Stem Cells: a Phase II Multicenter Trial Recruiting NCT02318485 Phase 2
24 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
25 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
26 Topical Infliximab in Autoimmune Eyes With Keratoprosthesis Withdrawn NCT02126020 Phase 1, Phase 2 topical infliximab
27 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
28 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
29 Safety, Tolerability and Activity of BIVV009 in Healthy Volunteers and Patients With Complement-mediated Disorders. A Single/Multiple Ascending Dose Phase 1 Study. Recruiting NCT02502903 Phase 1 BIV009
30 Topical Infliximab for the Treatment of Sterile Corneal Melt Recruiting NCT02987686 Phase 1 Topical Infliximab
31 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Unknown status NCT02874079
32 Explore the Mechanisms of Pruritus in Bullous Pemphigoid Patients During Remission Unknown status NCT01265082
33 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)
34 An Open Randomized Comparative Trial of Four Different Dressings for Cutaneous Coverage in Pemphigus and Pemphigoid Unknown status NCT02365675
35 Identification of Vulnerability Factors in the Course of Pemphigus Patients - SHS Pemphigus Clinical Trial Unknown status NCT02237313
36 Serum Free Fatty Acids Desaturase Activity Indices in Sera of Patients on Long-term High-dose Corticosteroid Treatment Unknown status NCT02872584 Prednisone
37 Impact of Neurological Diseases on the Prognosis of Bullous Pemphigoid: A Retrospective Study of 178 Patients Completed NCT03320798
38 Clinical Characteristics of Pruritus and Evaluation of Quality of Life in Patients With Bullous Pemphigoid Completed NCT03272958
39 Interest of Dosage of Anti-PB230, Anti-PB180 and Cytokines for Monitoring of Patients Suffering From Bullous Pemphigoid Completed NCT02883894
40 Pruritus and Pemphigoid in Nursing Home Patients Completed NCT02823067
41 Comparison of Two Therapeutic Strategies of Dermoval in Treatment of Bullous Pemphigus Completed NCT00213421 Dermoval
42 Register of Autoimmune Bullous Dermatoses Completed NCT02899923
43 A Controlled Cross Sectional Study: Prevalence of Cutaneous Autoimmune Phenomena in HIV Infected Patients Completed NCT03129308
44 A Study of the Association of Sleep Dysfunction and Burning Mouth Syndrome Completed NCT00862576
45 Systemic Immunosupressive Therapy for Eye Diseases (SITE) Cohort Study Completed NCT00116090
46 Evaluation of Immune Responses to Different Antigens in Non Infectious Ocular Inflammatory Diseases Completed NCT00357071
47 Clinical Effects and Safety of 3% Diquafosol Ophthalmic Solution for Patients With Dry Eye After Cataract Surgery: A Randomized Controlled Trial Completed NCT02608489 Diquafosol (Diquas);Sodium Hyaluronate (Hyalein)
48 Evaluation of Cytokines and Immunoglobulins in Serum and Blister Fluids Appeared Before Treatment and Subsequently Under Treatment in Bullous Pemphigoid Recruiting NCT03856840
49 Prevalence and Clinical Severity of Autoimmune Blistering Diseases Recruiting NCT02753777
50 Dipeptidyl Peptidase-IV Inhibitors, Risk Factor for Development of Bullous Pemphigoid? : Multicenter Retrospective Study in France and Switzerland Recruiting NCT03636763 data report

Search NIH Clinical Center for Bullous Pemphigoid

Inferred drug relations via UMLS 72 / 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:

41
Skin, Eye, T Cells, Testes, Neutrophil, B Cells, Breast

Publications for Bullous Pemphigoid

Articles related to Bullous Pemphigoid:

(show top 50) (show all 6308)
# Title Authors PMID Year
1
C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin. 9 38
19863700 2010
2
Serum anti-BPAG1 auto-antibody is a novel marker for human melanoma. 9 38
20479946 2010
3
Sequential intramolecular epitope spreading of humoral responses to human BPAG2 in a transgenic model. 9 38
19812601 2010
4
Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid. 9 38
19751242 2010
5
Immunoglobulin A antibodies against desmoglein 1, envoplakin, periplakin and BP230 in a patient with atypical bullous pemphigoid. 9 38
20507390 2010
6
Plakins in striated muscle. 9 38
19768769 2010
7
Anti-bullous pemphigoid 180 and 230 antibodies in a sample of unaffected subjects. 9 38
20083688 2010
8
Prevalence of autoantibodies to bullous pemphigoid antigens within the normal population. 9 38
20083697 2010
9
Conjunctival interleukin-13 expression in mucous membrane pemphigoid and functional effects of interleukin-13 on conjunctival fibroblasts in vitro. 9 38
19910508 2009
10
Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. 9 38
19737550 2009
11
Basement membrane changes in lichen planopilaris. 9 38
19522713 2009
12
Experimental animal models of bullous pemphigoid. 9 38
19755945 2009
13
Plasmin plays a role in the in vitro generation of the linear IgA dermatosis antigen LADB97. 9 38
19158842 2009
14
Pemphigoid gestationis: clinical and laboratory evaluation. 9 38
19936176 2009
15
Cicatrising conjunctivitis with anti-basement membrane autoantibodies in ectodermal dysplasia. 9 38
18815422 2008
16
Calcineurin/NFAT-dependent regulation of 230-kDa bullous pemphigoid antigen (BPAG1) gene expression in normal human epidermal keratinocytes. 9 38
18353617 2008
17
Expression of vinculin in autoimmune cutaneous diseases. 9 38
18327013 2008
18
Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. 9 38
18289320 2008
19
Serum levels of IgE anti-BP180 and anti-BP230 autoantibodies in patients with bullous pemphigoid. 9 38
17920818 2008
20
Enzyme-linked immunosorbent assay in autoimmune blistering diseases: preliminary experience of the Dermatology Department of Cagliari. 9 38
18833046 2008
21
Role of BP180NC16a-enzyme-linked immunosorbent assay (ELISA) in the diagnosis of bullous pemphigoid in China. 9 38
18173596 2008
22
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. 9 38
17611576 2007
23
Relative risk for cancer in mucous membrane pemphigoid associated with antibodies to the beta4 integrin subunit. 9 38
17524068 2007
24
Experimental models for the autoimmune and inflammatory blistering disease, Bullous pemphigoid. 9 38
17879094 2007
25
[Comparison of urinary proteomics between steroid-sensitive and steroid-resistant minimal change nephrotic syndrome in children]. 9 38
17959526 2007
26
Significance of IgG4 in the diagnosis of mucous membrane pemphigoid. 9 38
17344074 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. 9 38
17697150 2007
28
14-3-3 sigma isoform interacts with the cytoplasmic domain of the transmembrane BP180 in keratinocytes. 9 38
17443672 2007
29
Paraneoplastic pemphigus. 9 38
17683379 2007
30
Clinical and immunopathologic findings in oral lichen planus pemphigoides. 9 38
17531940 2007
31
[Paraneoplastic pemphigus]. 9 38
17513251 2007
32
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. 9 38
17263817 2007
33
Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases. 9 38
17222220 2007
34
Oral pemphigoid autoantibodies preferentially target BP180 ectodomain. 9 38
17141573 2007
35
Structural analysis of the plakin domain of bullous pemphigoid antigen1 (BPAG1) suggests that plakins are members of the spectrin superfamily. 9 38
17161423 2007
36
Relationship between cancer and oral pemphigoid patients with antibodies to alpha6-integrin. 9 38
17181734 2007
37
Antigen specificity in subsets of mucous membrane pemphigoid. 9 38
16810295 2006
38
Localisation of bullous pemphigoid antigen 180 (BP180) in cultured human keratinocytes: functionally relevant modification by calcium. 9 38
16906396 2006
39
Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum values. 9 38
17026501 2006
40
Clinical and diagnostic features of mucous membrane pemphigoid. 9 38
17052040 2006
41
Autoimmune subepidermal blistering diseases in Uganda: correlation of autoantibody class with age of patients. 9 38
16961507 2006
42
Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus vulgaris. 9 38
16882171 2006
43
Thymus and activation regulated chemokine (TARC)/CCL17 and skin diseases. 9 38
16859899 2006
44
Comparison of reactivity and epitope recognition between sera from American and Italian patients with oral pemphigoid. 9 38
16792670 2006
45
IgG4 as the predominant IgG subclass in pemphigoides gestationis. 9 38
16630180 2006
46
Epitopes in the linker subdomain region of envoplakin recognized by autoantibodies in paraneoplastic pemphigus patients. 9 38
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. 9 38
16512878 2006
48
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. 9 38
16424229 2006
49
Autoreactive T and B cells from bullous pemphigoid (BP) patients recognize epitopes clustered in distinct regions of BP180 and BP230. 9 38
16424234 2006
50
New insights into the autoantibody-mediated mechanisms of autoimmune bullous diseases and urticaria. 9 38
16466621 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 30)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.77 TNFSF13B LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
2
Show member pathways
13.31 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
3
Show member pathways
12.99 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
4
Show member pathways
12.81 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
5
Show member pathways
12.79 TNFSF13B RNASE3 IL5 HLA-DRB1 HLA-DQB1 CCL11
6 12.75 LAMC2 LAMB3 LAMA3 ITGA6 IL5
7
Show member pathways
12.72 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
8
Show member pathways
12.58 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
9
Show member pathways
12.41 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
10
Show member pathways
12.39 PPL EVPL DSP DSG3 DSG1
11
Show member pathways
12.31 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
12
Show member pathways
12.18 PLEC LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
13
Show member pathways
12.07 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
14
Show member pathways
12.07 LAMC2 LAMB3 LAMA3 ITGA6 HLA-DRB1 HLA-DQB1
15
Show member pathways
12.02 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6
16 11.94 PLEC ITGB4 ITGA6 DSP
17
Show member pathways
11.88 PLEC DSP DSG3 DSG1
18
Show member pathways
11.84 PPL PLEC EVPL DST DSP
19 11.83 LAMC2 LAMB3 LAMA3
20 11.78 TNFSF13B HLA-DRB1 HLA-DQB1
21 11.78 ITGA6 IL5 HLA-DRB1 HLA-DQB1
22 11.75 HLA-DRB1 HLA-DQB1 DSG1
23 11.74 LAMC2 LAMB3 LAMA3 ITGA6
24 11.59 IL5 DSP CCL11
25 11.59 IL5 CCL17 CCL11
26 11.57 LAMC2 LAMB3 LAMA3
27 11.42 IL5 CCL17 CCL11
28 11.19 ITGB4 ITGA6 COL17A1
29 11.13 TNFSF13B IL5 CCL17 CCL11
30 10.23 TNFSF13B LAMC2 LAMB3 LAMA3 ITGB4 ITGA6

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 cell junction GO:0030054 9.97 PPL PLEC ITGB4 EVPL DST DSP
2 intermediate filament GO:0005882 9.77 PPL PLEC EVPL DST DSP
3 cornified envelope GO:0001533 9.65 PPL EVPL DSP DSG3 DSG1
4 basement membrane GO:0005604 9.63 LAMC2 LAMB3 LAMA3 ITGA6 DST COL17A1
5 MHC class II protein complex GO:0042613 9.48 HLA-DRB1 HLA-DQB1
6 laminin-5 complex GO:0005610 9.4 LAMB3 LAMA3
7 desmosome GO:0030057 9.35 PPL EVPL DSP DSG3 DSG1
8 hemidesmosome GO:0030056 9.02 PLEC ITGB4 ITGA6 DST COL17A1
9 plasma membrane GO:0005886 10.23 TNFSF13B PPL PLEC ITGB4 ITGA6 HLA-DRB1
10 extracellular region GO:0005576 10.09 TNFSF13B RNASE3 LAMC2 LAMB3 LAMA3 IL5

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.93 TNFSF13B IL5 HLA-DRB1 HLA-DQB1 CCL17 CCL11
2 cell adhesion GO:0007155 9.91 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 DST
3 keratinization GO:0031424 9.83 PPL EVPL DSP DSG3 DSG1
4 cornification GO:0070268 9.8 PPL EVPL DSP DSG3 DSG1
5 extracellular matrix organization GO:0030198 9.8 LAMC2 LAMB3 LAMA3 ITGB4 ITGA6 COL17A1
6 wound healing GO:0042060 9.77 PPL PLEC EVPL DST DSP
7 integrin-mediated signaling pathway GO:0007229 9.73 ITGB4 ITGA6 DST
8 cell-matrix adhesion GO:0007160 9.72 ITGB4 ITGA6 COL17A1
9 skin development GO:0043588 9.67 ITGB4 ITGA6 DSP
10 epidermis development GO:0008544 9.63 LAMC2 LAMB3 LAMA3 EVPL DSP COL17A1
11 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.57 HLA-DRB1 HLA-DQB1
12 amelogenesis GO:0097186 9.54 ITGB4 ITGA6
13 nail development GO:0035878 9.52 ITGB4 ITGA6
14 humoral immune response mediated by circulating immunoglobulin GO:0002455 9.51 HLA-DRB1 HLA-DQB1
15 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 9.48 HLA-DRB1 HLA-DQB1
16 intermediate filament cytoskeleton organization GO:0045104 9.35 PPL PLEC EVPL DST DSP
17 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 9.71 PPL PLEC ITGA6 EVPL
2 structural constituent of cytoskeleton GO:0005200 9.54 PPL PLEC DSP
3 cytokine activity GO:0005125 9.46 TNFSF13B IL5 CCL17 CCL11
4 insulin-like growth factor I binding GO:0031994 9.32 ITGB4 ITGA6
5 MHC class II receptor activity GO:0032395 9.26 HLA-DRB1 HLA-DQB1
6 structural molecule activity GO:0005198 9.17 PPL PLEC LAMB3 LAMA3 EVPL DST
7 neuregulin binding GO:0038132 8.96 ITGB4 ITGA6

Sources for Bullous Pemphigoid

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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