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1.
Nat Commun ; 12(1): 302, 2021 01 12.
Artigo em Inglês | MEDLINE | ID: mdl-33436591

RESUMO

Pemphigoid diseases refer to a group of severe autoimmune skin blistering diseases characterized by subepidermal blistering and loss of dermal-epidermal adhesion induced by autoantibody and immune cell infiltrate at the dermal-epidermal junction and upper dermis. Here, we explore the role of the immune cell-secreted serine protease, granzyme B, in pemphigoid disease pathogenesis using three independent murine models. In all models, granzyme B knockout or topical pharmacological inhibition significantly reduces total blistering area compared to controls. In vivo and in vitro studies show that granzyme B contributes to blistering by degrading key anchoring proteins in the dermal-epidermal junction that are necessary for dermal-epidermal adhesion. Further, granzyme B mediates IL-8/macrophage inflammatory protein-2 secretion, lesional neutrophil infiltration, and lesional neutrophil elastase activity. Clinically, granzyme B is elevated and abundant in human pemphigoid disease blister fluids and lesional skin. Collectively, granzyme B is a potential therapeutic target in pemphigoid diseases.


Assuntos
Doenças Autoimunes/enzimologia , Doenças Autoimunes/patologia , Granzimas/antagonistas & inibidores , Granzimas/metabolismo , Animais , Autoantígenos/metabolismo , Vesícula , Quimiocina CXCL2/metabolismo , Fatores Quimiotáticos/farmacologia , Modelos Animais de Doenças , Epidermólise Bolhosa/enzimologia , Epidermólise Bolhosa/patologia , Humanos , Inflamação/patologia , Integrina alfa6/metabolismo , Interleucina-8/metabolismo , Infiltração de Neutrófilos/efeitos dos fármacos , Colágenos não Fibrilares/metabolismo , Penfigoide Bolhoso/enzimologia , Penfigoide Bolhoso/patologia , Índice de Gravidade de Doença
2.
Mol Immunol ; 126: 95-100, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32795664

RESUMO

Although T cells are considered as the central component in immune-mediated diseases, supportive evidence has demonstrated that B cells also contribute to the progression of these diseases. B cells are divided into various subsets according to their secreted cytokines. Different B cell subsets play diverse roles in immune-mediated dermatoses. Regulatory B cells (Bregs) are defined functionally by their ability to secrete IL-10, which has been revealed to contribute to immunological tolerance. Drugs that deplete B cells, such as rituximab, are now used for the treatment of several immune-mediated dermatoses. In this review, we present and discuss the current knowledge on the roles of B cells in several immune-mediate dermatoses including psoriasis, pemphigus, bullous pemphigoid, and dermatomyositis, atopic dermatitis.


Assuntos
Linfócitos B Reguladores/imunologia , Dermatite Atópica/imunologia , Dermatomiosite/imunologia , Penfigoide Bolhoso/imunologia , Pênfigo/imunologia , Psoríase/imunologia , Animais , Linfócitos B Reguladores/efeitos dos fármacos , Linfócitos B Reguladores/metabolismo , Dermatite Atópica/sangue , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/patologia , Dermatomiosite/sangue , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Desmogleína 3/metabolismo , Modelos Animais de Doenças , Humanos , Fatores Imunológicos/farmacologia , Fatores Imunológicos/uso terapêutico , Interleucina-10/metabolismo , Depleção Linfocítica/métodos , Penfigoide Bolhoso/sangue , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/patologia , Pênfigo/sangue , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Psoríase/sangue , Psoríase/tratamento farmacológico , Psoríase/patologia , Pele/imunologia , Pele/patologia
3.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao ; 42(2): 197-201, 2020 Apr 28.
Artigo em Chinês | MEDLINE | ID: mdl-32385025

RESUMO

Objective To investigate the clinical manifestations and laboratory characteristics of 6 cases of pemphigoid nodularis (PN). Method The clinical and laboratory data of 6 patients with PN admitted to the Department of Dermatology,Peking Union Medical College Hospital from January 2016 to August 2019 were retrospectively analyzed. Results PN mainly occurred in middle-aged and elderly people,with an average age of (58±16) years. Eosinophils were elevated in 4 patients. Immunoglobulin E (IgE) level was (530±672) kU/L in five patients. Direct immunofluorescence showed IgG and/or C3 deposition on basal membrane zone. Indirect immunofluorescence showed positive IgG anti-basement membrane zone,with a titer of 1:40-1:320. Enzyme-linked immunosorbent assay showed the anti-BP180 antibodies were positive [24-85 U/ml,average(43±26) U/ml] in 5 patients. None of the patients had neurological disorders. One patient was lost to follow-up. The disease recurred in 3 of 5 patients during the follow-up,and two patients still received maintenance corticosteroids. Conclusions PN mainly occurs in middle-aged and elderly individuals. It is featured by elevated eosinophils and total IgE and relatively low anti-BP180 antibody titers. Recurrence is common but PN is less likely to be associated with neurological diseases.


Assuntos
Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/patologia , Adulto , Idoso , Autoanticorpos/sangue , Ensaio de Imunoadsorção Enzimática , Eosinófilos , Humanos , Imunoglobulina E/sangue , Pessoa de Meia-Idade , Estudos Retrospectivos
6.
Acta Derm Venereol ; 100(5): adv00055, 2020 02 12.
Artigo em Inglês | MEDLINE | ID: mdl-32039458

RESUMO

Pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes. They are caused by autoantibodies targeting adhesion molecules located at the dermal-epidermal junction. While the diagnostics of pemphigoid diseases and insights into their pathogenesis have improved significantly, the development of novel treatments that are effective and safe remains an unmet medical need. However, numerous pre-clinical studies and early clinical trials have recently been launched. This review summarizes some pathways leading to drug development in pemphigoid diseases, namely: (i) hypothesis-driven drug development; (ii) omics-based drug development; (iii) drug repurposing; (iv) screening-based drug development; and (v) drug development based on careful clinical observations. Ultimately, it is hoped that this will lead to personalized and curative treatments.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Desenvolvimento de Medicamentos , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/imunologia , Proteínas Tirosina Quinases/administração & dosagem , Autoanticorpos/efeitos dos fármacos , Doenças Autoimunes/patologia , Moléculas de Adesão Celular/imunologia , Fumarato de Dimetilo/uso terapêutico , Doxiciclina/uso terapêutico , Epidermólise Bolhosa Adquirida/tratamento farmacológico , Epidermólise Bolhosa Adquirida/imunologia , Epidermólise Bolhosa Adquirida/patologia , Feminino , Previsões , Humanos , Masculino , Terapia de Alvo Molecular/métodos , Penfigoide Bolhoso/patologia , Dermatopatias Vesiculobolhosas/tratamento farmacológico , Dermatopatias Vesiculobolhosas/imunologia , Dermatopatias Vesiculobolhosas/patologia , Pesquisa Médica Translacional
8.
J Cutan Pathol ; 47(2): 121-127, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31603994

RESUMO

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disease associated with autoantibodies against BP180 and/or BP230 antigens. The immunoassays available for serological diagnostics include indirect immunofluorescence (IIF) on monkey esophagus (ME), salt-split skin (SSS), and enzyme-linked immunosorbent assay (ELISA) for BP180-NC16a and BP230. Only a few studies validated innovative BIOCHIP mosaic, but none compared agreement between BIOCHIP substrates with conventional methods separately. METHODS: We evaluated the agreement between BIOCHIP and conventional methods and assessed sensitivity and specificity in BP diagnosis. The study comprised 51 BP patients and 39 controls. RESULTS: Analysis showed very good agreement between BIOCHIP-SSS vs classic IIF-SSS (0.933, P < 0.001) and for BIOCHIP-BP180-NC16a vs ELISA-BP180-NC16a (0.933, P < 0.001). A good strength of agreement between BIOCHIP-ME vs classic IIF-ME was observed (0.694, P < 0.001) similar to BIOCHIP-BP230 vs ELISA-BP230 (0.793, P < 0.001). BIOCHIP-ME sensitivity was 51.0%, whereas IIF-ME was 76.5%. Epidermal reaction on BIOCHIP-SSS was found in 94.1% of BP patients and in all patients on IIF-SSS (sensitivity 100%). BIOCHIP-BP180-NC16a sensitivity was lower than in ELISA-BP180-NC16a (76.5% vs 82.4%). BP230 sensitivity of both methods was similar (45.1% vs 43.1%). The specificity for all antigens was 100%. CONCLUSION: BIOCHIP mosaic is a useful method presenting satisfactory agreement with conventional immunoassays.


Assuntos
Penfigoide Bolhoso , Idoso , Ensaio de Imunoadsorção Enzimática , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Masculino , Pessoa de Meia-Idade , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/patologia , Sensibilidade e Especificidade
9.
Int J Dermatol ; 59(2): 197-206, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31605541

RESUMO

BACKGROUND: Bullous pemphigoid (BP) has been associated with dipeptidyl peptidase-4 inhibitors (DPP4i). Clinical features, outcomes, and risk of BP for new DPP4i (linagliptin, saxagliptin, and alopgliptin) are not well established. Comparison of risk of BP appearance for DPP4i and other oral antidiabetic drugs (OADs) such as sodium glucose cotransporter 2 inhibitors has not been studied to date. OBJECTIVES: To describe the prevalence, sociodemographic, clinical, and histopathological characteristics, and outcome after drug withdrawal in DPP-4i-associated BP cases from our hospital. To review all Spanish spontaneous notifications of BP where DPP4i or OADs were included as suspected drugs and calculate the reporting odds ratios (RORs). METHODS: A retrospective observational study was performed examining the association between DDP4i and BP. Clinical features and RORs were analyzed. Data from the Spanish Pharmacovigilance System (SEFV) are included. RESULTS: In our center, 28 of 89 patients with BP (31.5%) were under DPP4i treatment; 53.6% were male, and mean age was 80.8 years. BP debuted the first year after DPP4i in 57.2%. BP control was achieved within 3.7 months after drug withdrawal. Regarding SEFV, 22 of 972 spontaneous notifications were related to BP and DPP4i. RORs were superior for DPP4i compared to other OADs. Vildagliptin had the highest ROR. CONCLUSIONS: We present the largest DPP4i-induced BP case series in a single center, with a detailed study of the sociodemographic, clinical, and histopathological characteristics of each patient, and their treatment and outcome. Vildagliptin had the highest risk. DPP4i-associated BP does not seem to have specific clinical characteristics.


Assuntos
Inibidores da Dipeptidil Peptidase IV/efeitos adversos , Penfigoide Bolhoso/induzido quimicamente , Idoso , Idoso de 80 Anos ou mais , Bases de Dados Factuais , Feminino , Humanos , Linagliptina/efeitos adversos , Masculino , Pessoa de Meia-Idade , Penfigoide Bolhoso/sangue , Penfigoide Bolhoso/patologia , Farmacovigilância , Estudos Retrospectivos , Fosfato de Sitagliptina/efeitos adversos , Inibidores do Transportador 2 de Sódio-Glicose/efeitos adversos , Espanha , Vildagliptina/efeitos adversos
11.
Exp Mol Pathol ; 112: 104331, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31705881

RESUMO

BACKGROUND: Significant alterations of the cutaneous microbiota (CM) have been recently demonstrated in bullous pemphigoid (BP). Microbiome data of both oral cavity (OM) and gut (GM) from patients affected by bullous disease are not available yet and, further consistent studies focused on the role of such microbial populations are still missing. OBJECTIVE: Objective: In this pilot study we characterized and compared GM, OM and CM of patients affected by pemphigus vulgaris (PV) and BP to investigate a distinctive microbiome composition in this two rare dermatological disorders. METHODS: High-throughput sequencing of the V1-V3 hyper-variable regions of 16S rRNA was used to compare the bacterial community composition of stool, skin and oral mucosae swabs in a cohort of PV and BP patients. A dedicated bioinformatics software coupled with in-house pipeline was implemented to analyse and compare diseases dataset. RESULTS: GM samples of both PV and BP patients were principally characterized by Firmicutes and Bacteroidetes phyla. Interestingly, the Firmicutes phylum and Staphylococcus genus were mainly represented in cutaneous samples. The diversity of phyla in oral mucosae was higher than those of gut and skin samples and, Bacteroidetes phylum was significantly underrepresented in all PV samples. CONCLUSION: Firmicutes phylum and Staphilococcus genus were the most represented in OM and CM swabs of PV and BP microbial populations. Moreover, we argue the quantitative imbalance linked to the decrease of Bacteriodetes in the oral cavity of PV patients might be associated to disease typical fetor. To shed light on this peculiar feature further studies are still required.


Assuntos
Microbioma Gastrointestinal/genética , Penfigoide Bolhoso/genética , Pênfigo/genética , Pele/microbiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Bacteroidetes/genética , Bacteroidetes/isolamento & purificação , Feminino , Firmicutes/genética , Firmicutes/isolamento & purificação , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Microbiota/genética , Pessoa de Meia-Idade , Boca/metabolismo , Boca/microbiologia , Penfigoide Bolhoso/microbiologia , Penfigoide Bolhoso/patologia , Pênfigo/microbiologia , Pênfigo/patologia , RNA Ribossômico 16S/genética , Pele/metabolismo
13.
Front Immunol ; 10: 2466, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31695695

RESUMO

The many clinical aspects of anti-p200 pemphigoid are not well-characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase, and Web of Science databases (1900-2018). Case reports and series of patients were included. A total of 68 eligible studies that comprised 113 anti-p200 pemphigoid patients were included in the qualitative analysis, where there was a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, and 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%), were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than in the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.


Assuntos
Autoanticorpos/imunologia , Laminina/imunologia , Penfigoide Bolhoso , Psoríase , Idade de Início , Idoso , Feminino , Humanos , Masculino , Penfigoide Bolhoso/epidemiologia , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/patologia , Psoríase/epidemiologia , Psoríase/imunologia , Psoríase/patologia
15.
Dermatol Online J ; 25(10)2019 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-31735006

RESUMO

Immune checkpoint inhibitors are used to treat numerous malignancies but may be associated with severe adverse events. Bullous dermatoses, chiefly bullous pemphigoid (BP), are potentially progressive adverse events that cause blistering skin lesions and may involve a significant body surface area. Herein, we report an 87-year-old man with urothelial cell carcinoma undergoing atezolizumab treatment who presented with an acute-onset blistering eruption. Biopsy revealed a subepidermal bulla, direct immunofluorescence revealed linear IgG and C3 deposits at the dermal-epidermal junction, and serum studies revealed elevated levels of antibodies to BP180 and BP230. Anti-PD-L1-induced BP was diagnosed, immunotherapy was withheld, and he was treated with oral doxycycline with niacinamide and clobetasol ointment. He restarted atezolizumab and has successfully received four cycles (every 3 weeks) while continuing this BP treatment regimen. A literature review revealed eight other cases of anti-PD-L1-induced bullous disorders. The incidence of bullous dermatoses with anti-PD-1/anti-PD-L1 agents combined is 1%, whereas the reported incidence for anti-PD-L1 agents alone ranges from 1.3-5%, raising concerns for a higher overall risk. In addition to our case, only one other case reported successful resumption of immunotherapy. Early control and management of immunotherapy-induced BP may reduce complications and prevent treatment discontinuation.


Assuntos
Anticorpos Monoclonais Humanizados/efeitos adversos , Penfigoide Bolhoso/induzido quimicamente , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Idoso de 80 Anos ou mais , Humanos , Imunoterapia/efeitos adversos , Masculino , Penfigoide Bolhoso/patologia
17.
BMJ Case Rep ; 12(9)2019 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-31570343

RESUMO

Dipeptidyl peptidase 4 (DPP-4) inhibitors are increasingly used these days in management of diabetes. There has been reported in a few case reports of increasing association between DPP-4 inhibitor use and bullous pemphigoid (BP). We report a case of association between linagliptin use and BP and subsequent treatment with rituximab.


Assuntos
Inibidores da Dipeptidil Peptidase IV/efeitos adversos , Erupção por Droga/patologia , Linagliptina/efeitos adversos , Penfigoide Bolhoso/induzido quimicamente , Rituximab/uso terapêutico , Idoso , Antibacterianos/uso terapêutico , Clobetasol/uso terapêutico , Inibidores da Dipeptidil Peptidase IV/administração & dosagem , Erupção por Droga/tratamento farmacológico , Humanos , Linagliptina/administração & dosagem , Masculino , Minociclina/uso terapêutico , Penfigoide Bolhoso/patologia , Prednisona/uso terapêutico , Resultado do Tratamento
18.
Acta Dermatovenerol Croat ; 27(3): 184-187, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31542063

RESUMO

Bullous pemphigoid (BP) is an autoimmune disorder which is usually chronic, with blistering that predominantly affects the skin and occasionally the mucosa, and which includes several different types. One of them is a very rare dyshidrosiform type which is localized on the hands and feet with small or large blisters on the palmoplantar surfaces. BP resulting from a drug reaction is a relatively rare occurrence, and so far more than 50 different medications have been identified as triggers. The aim of this article was to present the case of a paraplegic patient who developed this rare dyshidrosiform type of BP while he was being neurologically treated with baclofen. In spite of therapy with systemic and topical corticosteroids and other measures, successful treatment was achieved only after eliminating baclofen from the patient's regimen. His general state of health was seriously endangered due to nasal and skin methicillin-resistant Staphylococcus aureus (MRSA), urinary infection, and oral mycosis (soor), and he was at high risk of sepsis and a fatal outcome. Through our efforts, however, we managed to achieve an excellent outcome. According to our knowledge, this was the first case of baclofen-induced dyshidrosiform BP.


Assuntos
Baclofeno/efeitos adversos , Staphylococcus aureus Resistente à Meticilina , Relaxantes Musculares Centrais/efeitos adversos , Penfigoide Bolhoso/induzido quimicamente , Infecções Estafilocócicas/complicações , Infecções Urinárias/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Paraplegia/complicações , Penfigoide Bolhoso/patologia
19.
J Dtsch Dermatol Ges ; 17(10): 1039-1051, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31562692

RESUMO

Eosinophilic dermatoses are a heterogeneous group of diseases, characterized by an eosinophil-rich infiltrate and/or degranulation of eosinophils. Blood eosinophilia may be an associated feature. Typical, albeit not specific histological findings include 'flame figures', which are caused by the accumulation of cationic proteins released by eosinophils and subsequent collagen denaturation. "Classic" eosinophilic dermatoses include eosinophilic cellulitis (Wells syndrome), granuloma faciale, eosinophilic fasciitis (Shulman syndrome) and eosinophilic folliculitis (Ofuji disease). In addition, there is a multitude of skin diseases that present with varying degrees of eosinophilic infiltration. These include atopic dermatitis, bullous pemphigoid, urticaria, allergic contact dermatitis, prurigo nodularis, arthropod bite reaction, parasitic infections, and drug hypersensitivity. Even though these disorders share a common characteristic (tissue eosinophilia), they differ greatly in their clinical presentation.


Assuntos
Colágeno/metabolismo , Proteína Catiônica de Eosinófilo/metabolismo , Eosinófilos/imunologia , Dermatopatias/imunologia , Celulite (Flegmão)/tratamento farmacológico , Celulite (Flegmão)/imunologia , Celulite (Flegmão)/patologia , Dermatite Alérgica de Contato/tratamento farmacológico , Dermatite Alérgica de Contato/imunologia , Dermatite Alérgica de Contato/patologia , Dermatite Atópica/tratamento farmacológico , Dermatite Atópica/imunologia , Dermatite Atópica/patologia , Hipersensibilidade a Drogas/tratamento farmacológico , Hipersensibilidade a Drogas/imunologia , Hipersensibilidade a Drogas/patologia , Eosinofilia/tratamento farmacológico , Eosinofilia/imunologia , Eosinofilia/patologia , Eosinófilos/patologia , Eosinófilos/ultraestrutura , Fasciite/tratamento farmacológico , Fasciite/imunologia , Fasciite/patologia , Foliculite/tratamento farmacológico , Foliculite/imunologia , Foliculite/patologia , Granuloma/tratamento farmacológico , Granuloma/imunologia , Granuloma/patologia , Humanos , Mordeduras e Picadas de Insetos/tratamento farmacológico , Mordeduras e Picadas de Insetos/imunologia , Mordeduras e Picadas de Insetos/patologia , Doenças Parasitárias/tratamento farmacológico , Doenças Parasitárias/imunologia , Doenças Parasitárias/patologia , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/patologia , Prurigo/tratamento farmacológico , Prurigo/imunologia , Prurigo/patologia , Dermatopatias/classificação , Dermatopatias/tratamento farmacológico , Dermatopatias/patologia , Dermatopatias Vesiculobolhosas/tratamento farmacológico , Dermatopatias Vesiculobolhosas/imunologia , Dermatopatias Vesiculobolhosas/patologia , Urticária/tratamento farmacológico , Urticária/imunologia , Urticária/patologia
20.
JCI Insight ; 4(15)2019 08 08.
Artigo em Inglês | MEDLINE | ID: mdl-31391346

RESUMO

The treatment of most autoimmune diseases still relies on systemic immunosuppression and is associated with severe side effects. The development of drugs that more specifically abrogate pathogenic pathways is therefore most desirable. In nature, such specificity is exemplified, e.g., by the soft tick-derived biotherapeutic Coversin, which locally suppresses immune responses by inhibiting complement factor 5 (C5) and leukotriene B4 (LTB4). C5a, a proteolytic fragment of C5, and LTB4 are critical drivers of skin inflammation in pemphigoid diseases (PDs), a group of autoimmune blistering skin diseases. Here, we demonstrate that both Coversin and its mutated form L-Coversin, which inhibits LTB4 only, dose dependently attenuate disease in a model of bullous pemphigoid-like epidermolysis bullosa acquisita (BP-like EBA). Coversin, however, reduces disease more effectively than L-Coversin, indicating that inhibition of C5 and LTB4 synergize in their suppressing effects in this model. Further supporting the therapeutic potential of Coversin in humans, we found that C5a and LTB4 are both present in the blister fluid of patients with BP in quantities inducing the recruitment of granulocytes and that the number of cells expressing their receptors, C5aR1 and BLT1, respectively, is increased in perilesional skin. Collectively, our results highlight Coversin and possibly L-Coversin as potential therapeutics for PDs.


Assuntos
Produtos Biológicos/farmacologia , Complemento C5/antagonistas & inibidores , Inativadores do Complemento/farmacologia , Leucotrieno B4/antagonistas & inibidores , Penfigoide Bolhoso/tratamento farmacológico , Animais , Produtos Biológicos/uso terapêutico , Células Cultivadas , Quimiotaxia/efeitos dos fármacos , Quimiotaxia/imunologia , Colágeno Tipo VII/administração & dosagem , Colágeno Tipo VII/imunologia , Complemento C5/imunologia , Complemento C5/metabolismo , Inativadores do Complemento/uso terapêutico , Modelos Animais de Doenças , Granulócitos/imunologia , Voluntários Saudáveis , Humanos , Leucotrieno B4/imunologia , Leucotrieno B4/metabolismo , Masculino , Camundongos , Neutrófilos , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/patologia , Cultura Primária de Células , Coelhos , Pele/imunologia , Pele/patologia
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