Raras
Buscar doenças, sintomas, genes...
Pênfigo vulgar
ORPHA:704CID-10 · L10.0CID-11 · EB40.0OMIM 169610DOENÇA RARA

O pênfigo é um grupo de doenças cutâneas autoimunes crônicas caracterizadas por formações de bolhas na camada externa da pele e nas membranas mucosas. Foram caracterizadas três formas clínicas, sendo o pênfigo vulgar a mais frequente (75%).

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

O pênfigo é um grupo de doenças cutâneas autoimunes crônicas caracterizadas por formações de bolhas na camada externa da pele e nas membranas mucosas. Foram caracterizadas três formas clínicas, sendo o pênfigo vulgar a mais frequente (75%).

Pesquisas ativas
8 ensaios
45 total registrados no ClinicalTrials.gov
Publicações científicas
4.830 artigos
Último publicado: 2026 Apr 15

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
18.0
Europe
Início
Adult
+ childhood, elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: L10.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧬
Pele e cabelo
4 sintomas
📏
Crescimento
3 sintomas
🛡️
Imunológico
3 sintomas
🧠
Neurológico
2 sintomas

+ 10 sintomas em outras categorias

Características mais comuns

90%prev.
Cicatrização atípica da pele
Muito frequente (99-80%)
90%prev.
Acantólise
Muito frequente (99-80%)
90%prev.
Perda de peso
Muito frequente (99-80%)
90%prev.
Urticária
Muito frequente (99-80%)
90%prev.
Bolhas na mucosa oral
Muito frequente (99-80%)
90%prev.
Autoimunidade
Muito frequente (99-80%)
22sintomas
Muito frequente (10)
Frequente (7)
Ocasional (2)
Muito raro (1)
Sem dados (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 22 características clínicas mais associadas, ordenadas por frequência.

Cicatrização atípica da peleAtypical scarring of skin
Muito frequente (99-80%)90%
AcantóliseAcantholysis
Muito frequente (99-80%)90%
Perda de pesoWeight loss
Muito frequente (99-80%)90%
UrticáriaUrticaria
Muito frequente (99-80%)90%
Bolhas na mucosa oralOral mucosal blisters
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico4.830PubMed
Últimos 10 anos200publicações
Pico2025137 papers
Linha do tempo
2026Hoje · 2026🧪 1996Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
3Fase 33
2Fase 25
1Fase 12
·Pré-clínico9
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Pênfigo vulgar

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

45 ensaios clínicos encontrados, 8 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
1.841 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.841

#1

Iatrogenic Upper Gastrointestinal Bleeding Following Nasogastric Tube Insertion in a Patient with Pemphigus Vulgaris.

The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology2026 Jan 05

Cite this article as: Ketenci YB, .akır A, Yılmaz GA, Avcıoğlu U, Demir.z H, Akca M. Iatrogenic upper gastrointestinal bleeding following nasogastric tube insertion in a patient with pemphigus vulgaris. Turk J Gastroenterol. 2026;37(3):403-405.

#2

Caspase-activation powers anti-Desmoglein 3-induced acantholysis in human epidermis.

Cell death discovery2026 Feb 19

Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease caused by circulating autoantibodies against desmoglein (Dsg) 1 and Dsg 3. Whether acantholysis in PV results exclusively from antibody binding to Dsgs or involves additional factors remains controversial. Given that Fas-Ligand (FasL), an activator of apoptotic caspase-8, is increased in the serum and the skin of patients with PV, we investigated the role of caspases in anti-Dsg3-mediated acantholysis using both ex vivo and in vitro models. Our results demonstrated that anti-Dsg3 antibodies induced acantholysis ex vivo in the absence of caspase activation, primarily through the redistribution of Dsg3 to intracellular compartments. FasL-induced caspase activation led to a synergistic amplification of anti-Dsg3-mediated loss of cell adhesion by promoting Dsg3 cleavage. This dual mechanism provides new insights into the disease heterogeneity of PV.

#3

[Value of direct immunofluorescence in the diagnosis of oral mucosal pemphigus vulgaris: A retrospective study based on multi-index combined analysis].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences2026 Feb 18

To clarify the diagnostic efficacy of various diagnostic methods through multi-index combined analysis, and to explore the core diagnostic value of direct immunofluorescence (DIF) in oral mucosal pemphigus vulgaris (PV). A total of 53 patients with confirmed oral mucosal PV were included, retrospectively. Their data of DIF, histopathology, serum enzyme-linked immunosorbent assay (ELISA) and clinical diagnosis were systematically analyzed, and diagnostic efficacy of each method was evaluated using indicators, such as sensitivity, specificity, and area under curve (AUC) of the receiver operating characteristic (ROC) curve. The results showed that among the 53 patients, middle-aged and elderly females were predominant, the buccal mucosa was the most common involved site, and most patients had a history of blistering. The positive rate of DIF was 96.23%, mainly manifested as a reticular fluorescent deposition between epithelial spinous cells, demonstrating the highest diagnostic value with a sensitivity of 96.23%, specificity of 100.00%, and AUC of 0.981. Histopathology ranked second, with a sensitivity of 94.34%, a specificity of 100.00%, and an AUC value of 0.972. The ELISA test had a sensitivity of 82.61%, a specificity of 82.35%, and an AUC value of 0.825. Although the sensitivity of clinical diagnosis was acceptable, its specificity was relatively low. Additionally, DIF exhibited complementarity with histopathology, ELISA, and clinical diagnosis, and combined testing could improve diagnostic accuracy. DIF is the "gold standard" for the diagnosis of oral mucosal PV. A comprehensive diagnostic workflow of "clinical manifestation-DIF-histopathology-ELISA" is proposed. This integrated diagnostic system not only significantly improves the accuracy of oral mucosal PV diagnosis but also aligns with the core principles of precision medicine, providing a basis for indivi-dualized treatment. 基于多指标联合分析,探讨直接免疫荧光(direct immunofluorescence,DIF)对口腔黏膜寻常型天疱疮(pemphigus vulgaris,PV)的诊断效能。 回顾性分析53例确诊口腔黏膜PV的患者,收集患者临床资料及DIF、组织病理学、血清学酶联免疫吸附试验(enzyme-linked immunosorbnent assay,ELISA)检测结果,采用灵敏度、特异度、受试者工作特征(receiver operating characteristic,ROC)曲线的曲线下面积(area under curve,AUC)等指标评估各方法的诊断效能。 患者以中老年女性为主,颊黏膜为最常见受累部位,大部分患者伴有起疱史。DIF检测的阳性率为96.23%,主要表现为上皮棘细胞间网状绿色荧光沉积,其诊断价值最高,灵敏度为96.23%,特异度为100.00%,AUC值为0.981;组织病理学次之,灵敏度为94.34%,特异度为100.00%,AUC值为0.972;ELISA检测的灵敏度为82.61%,特异度为82.35%,AUC值为0.825。DIF与组织病理学、ELISA及临床诊断具有互补性,联合检测可提高诊断准确性。 DIF是诊断口腔黏膜PV的核心指标,建议采用“临床表现-DIF-组织病理学-ELISA”综合诊断流程;该综合诊断体系不仅能显著提升口腔黏膜PV诊断的精准度,也契合了精准医疗的核心理念,可为个性化治疗提供依据。

#4

3D organotypic skin models recapitulate autoantibody-driven pemphigus pathomechanisms and targeted therapeutic response.

Science advances2026 Jan 09

Advanced three-dimensional (3D) tissue models that recapitulate autoimmune disease progression can enable mechanistic studies and accelerate the development of targeted therapies with reduced reliance on systemic immunosuppression. Here, we present a dynamic biofabrication strategy combining 3D bioprinting and cell self-organization to create multilayered skin constructs with a stratified epidermis atop a vascularized, fibroblast-remodeled dermis. This platform reconstitutes native skin architecture to model pemphigus vulgaris, an autoantibody-driven blistering disorder. Tightly reassembled keratinocytes form cell-cell adhesions that replicate pathogenic antibody-induced disruption of the epidermal barrier, while embedded vasculature and fibroblasts shape dermal barriers that regulate molecular diffusion. We demonstrate that these tissue barriers define disease phenotypes and therapeutic responses by modulating antibody and drug penetration. As proof of concept, we quantitatively evaluate epidermal growth factor receptor-targeted inhibitors by measuring individual cell-cell junction integrity and applying machine learning-assisted image texture analysis. This organotypic skin model provides a physiomimetic testbed for precise investigation of autoimmune pathogenesis and high-throughput screening of targeted therapeutic agents.

#5

Epithelial Barrier Diseases Among Adult Patients With Seborrheic Dermatitis.

JAMA dermatology2026 Jan 01

The epithelial barrier theory (EBT) proposes that epithelial barrier disruption is implicated in the development of skin, respiratory, gastrointestinal, and ocular diseases (epithelial barrier diseases, or EBDs). There is a need to better understand the relationship between seborrheic dermatitis and EBDs, and we hypothesize that seborrheic dermatitis, characterized by epithelial barrier dysfunction, is associated with increased frequency of other EBDs. To explore the association between seborrheic dermatitis and EBDs. This retrospective cohort study used a large US administrative claims database, which included data collected from multiple health care centers and patient care settings across the US from January 1, 2016, through June 30, 2022. This study consisted of patients aged 18 years and older at enrollment, with at least 1 year of continuous enrollment, and with a minimum of 2 visits on unique days to a medical professional. The mean (SD) patient follow-up time was 3.46 (1.80) years with a total follow-up of more than 70 million person-years. Individuals with missing data for demographic covariates, including age, sex, and division (ie, billing region), were excluded. Data were analyzed from January to September 2025. Diagnosis of seborrheic dermatitis at any point during the observation period. Diagnosis of an EBD at any point during the observation period. Of 20 274 189 patients, 733 776 (3.62%) had seborrheic dermatitis (median [IQR] age, 62.63 [41.53-70.55] years; 54.7% female). Using adjusted models, seborrheic dermatitis was positively associated with atopic dermatitis (odds ratio [OR], 3.21; 95% CI, 3.18-3.24), alopecia areata (OR, 4.02; 95% CI, 3.93-4.11), contact dermatitis (OR, 2.25; 95% CI, 2.23-2.26), psoriasis (OR, 3.26; 95% CI, 3.23-3.29), rosacea (OR, 4.52; 95% CI, 4.49-4.56), hidradenitis suppurativa (OR, 1.63; 95% CI, 1.58-1.68), chronic spontaneous urticaria (OR, 1.35; 95% CI, 1.33-1.37), pemphigus vulgaris (OR, 1.48; 95% CI, 1.31-1.69), bullous pemphigoid (OR, 1.60; 95% CI, 1.51-1.70), rhinosinusitis (OR, 1.24; 95% CI, 1.24-1.25), celiac disease (OR, 1.36; 95% CI, 1.32-1.39), irritable bowel syndrome (OR, 1.32; 95% CI, 1.31-1.33), ocular allergy (OR, 1.39; 95% CI, 1.37-1.41), and dry eye (OR, 1.48; 95% CI, 1.48-1.49) and was negatively associated with chronic obstructive pulmonary disease (OR, 0.72; 95% CI, 0.71-0.72) and pulmonary hypertension (OR, 0.70; 95% CI, 0.69-0.71). These findings support the EBT as a shared driver in the pathogenesis of seborrheic dermatitis and other diverse EBDs and encourage further investigation into the underlying mechanisms of disease pathogenesis.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC2.989 artigos no totalmostrando 192

2026

Bullous pemphigoid and Graves' disease developing during prolonged remission of pemphigus vulgaris.

European journal of dermatology : EJD
2026

Experience with rituximab in patients with pemphigus vulgaris and hepatitis B.

Actas dermo-sifiliograficas
2026

A Single-Center Profile of Pemphigus in China: Significant Diagnostic Delay and Evolving Treatment Patterns.

Clinical, cosmetic and investigational dermatology
2026

Diagnostic Delay in Desquamative Gingivitis: An Observational Cohort Study.

Oral diseases
2026

Pemphigus Vulgaris With an Elevated Faecal Calprotectin Mimicking Inflammatory Bowel Disease: A Case Report.

Cureus
2026

Iatrogenic Upper Gastrointestinal Bleeding Following Nasogastric Tube Insertion in a Patient with Pemphigus Vulgaris.

The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology
2026

IL‑37/IL‑1R8 blocks keratinocyte acantholysis via suppressing ADAM17/EGFR.

International journal of molecular medicine
2026

Pesticide Exposure as a Risk Factor for Pemphigus Vulgaris: Findings from a Case-Control Study conducted in Liguria, Italy.

Clinical and experimental dermatology
2026

Pemphigus Vegetans: Insights from a systematic review of the literature.

Clinical and experimental dermatology
2025

What has Better Diagnostic Accuracy in Pemphigus? Direct Immunofluorescence of Plucked Hair, Oral Scrapes or Tzanck Smears!

Acta dermatovenerologica Croatica : ADC
2026

Diagnostic Accuracy of Anti-Desmoglein 1 and 3 Antibodies by Enzyme-Linked Immunosorbent Assay (ELISA) in Pemphigus Vulgaris.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2026

Could vector-derived cadherin mimicry contribute to pemphigus vulgaris? An immunogenetic and in silico study involving HLA-DRB104:02 and 14:01.

Frontiers in immunology
2026

Case Report: Pemphigus following COVID-19 mRNA vaccination: two cases achieving long-term remission without relapse.

Frontiers in immunology
2026

Autoimmune Bullous Diseases: Therapeutic Update.

Drugs
2026

A case of juvenile pemphigus vulgaris effectively treated with dupilumab.

JAAD case reports
2025

Corticosteroid-induced central serous chorioretinopathy in a pemphigus vulgaris patient.

Dermatology online journal
2025

Non-surgical treatment options for gingival recession: a dermatologic perspective.

Dermatology online journal
2026

A Specific Signature of Circulating Free Fatty Acid Discriminates Bullous Pemphigoid From Pemphigus Vulgaris and Healthy Controls.

Experimental dermatology
2026

Caspase-activation powers anti-Desmoglein 3-induced acantholysis in human epidermis.

Cell death discovery
2026

Pemphigus Vulgaris Mimicking Behçet's Syndrome With Oral and Vulvar Ulcers: A Case Report.

Cureus
2026

The Changing Trends in the Incidence and Clinical Presentation of Immunobullous Disorders Before and During the COVID-19 Era in a Tertiary Healthcare Centre in Andhra Pradesh.

Cureus
2026

Optimizing Local Treatment in Pemphigus and Pemphigoid: Current Evidence and Unmet Needs.

International journal of dermatology
2026

Dihydroartemisinin attenuates pemphigus vulgaris by regulating Dsg3 and the TLR9/NF-κB pathway.

American journal of translational research
2026

Correlation of platelet-related parameters and autoantibodies in patients with autoimmune bullous diseases.

Laboratory medicine
2026

Anti-Desmoglein 3 Avidity Correlates Better With Pemphigus Vulgaris Severity Than Anti-Desmoglein 3 Concentration.

International journal of dermatology
2026

Unmasking: paronychia as the hidden face of pemphigus.

Skin health and disease
2026

Navigating the Complexities of Pemphigus Vulgaris: A Comprehensive Iranian Study.

Immunity, inflammation and disease
2026

[Value of direct immunofluorescence in the diagnosis of oral mucosal pemphigus vulgaris: A retrospective study based on multi-index combined analysis].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2025

Telitacicept in combination with systemic steroid therapy in a patient with refractory pemphigus vulgaris and tuberculosis: a case report.

European journal of dermatology : EJD
2025

An Update on Pemphigus Vulgaris in Pregnancy and Neonates: Management Options and Our Clinical-Laboratory Experience.

Medicina (Kaunas, Lithuania)
2026

Autoimmune Disorders Affecting the Larynx: Review of Laryngoscopic Findings and Approach to Multidisciplinary Management.

The Annals of otology, rhinology, and laryngology
2026

Pemphigus Vulgaris Successfully Treated with Belimumab: A Case Report.

Indian journal of dermatology
2026

Signalling in pemphigus vulgaris: shedding light on the protein kinase C conundrum.

The British journal of dermatology
2025

Pemphigus vegetans successfully treated with systemic corticosteroid and rituximab: a rare case report with 15-month follow-up and literature review.

Frontiers in immunology
2026

Development of Autoimmune Bullous Disorders in Patients Exposed to Radiation Therapy: A Systematic Review.

Journal of cutaneous medicine and surgery
2026

Clinical Phenotype Transition in Pemphigus May Suggest Undertreatment with Rituximab: Findings from a Retrospective Single-Centre Analysis of Relapsed Patients.

Dermatology (Basel, Switzerland)
2026

Bullous Diseases in the Perioperative Setting: Anesthetic Considerations.

Cureus
2025

Pemphigus Revealing Profound Hypothyroidism: A Rare Association.

Cureus
2025

Vulgar Verruca or Seborrheic Keratosis: A Clinical Challenge in Tattooed Skin.

Cureus
2025

Pemphigus foliaceus transforming to pemphigus vulgaris: a case report.

Frontiers in medicine
2026

Oral Microbial Dysbiosis and Pathogenic Functional Shifts in Mucosa-Dominant Pemphigus Vulgaris and Low-Risk Mucous Membrane Pemphigoid.

Oral diseases
2026

Mechanistic and systemic perspectives on TWEAK in pemphigus vulgaris.

The British journal of dermatology
2026

Comparing the genetic and serum expression of LL-37 Antimicrobial Peptide in pemphigus vulgaris and pemphigus foliaceus patients.

Anais brasileiros de dermatologia
2026

BLyS/APRIL dual inhibition by telitacicept for refractory pemphigus vulgaris combined with hepatocellular cancer.

Anais brasileiros de dermatologia
2025

Esophagitis Dissecans Superficialis Associated With Mucosal Pemphigus vulgaris: A Rare and Underrecognized Clinical Presentation.

Cureus
2026

3D organotypic skin models recapitulate autoantibody-driven pemphigus pathomechanisms and targeted therapeutic response.

Science advances
2026

Noninvasive Imaging Techniques as Modern Diagnostic Tools in Desquamative Gingivitis: Focus on RCM, OCT, and LC-OCT.

Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI)
2026

Persistence of anti-Desmoglein 3 Antibodies in Patients with Pemphigus Vulgaris in Clinical Remission is Associated with Restricted Isotype Diversity and Desmoglein 3 Ectodomain Recognition.

The British journal of dermatology
2025

Emerging Treatments in Pemphigus: Is Healing an Achievable Goal?

American journal of clinical dermatology
2025

Clinical correlation of anti-desmoglein antibody dynamics in pemphigus treated with rituximab.

Frontiers in immunology
2026

Isolated perineal plaque as the initial presentation of pemphigus vulgaris.

JAAD case reports
2026

Rituximab resistant pemphigus vulgaris successfully treated with obinutuzumab.

JAAD case reports
2025

Acantholytic Acanthoma Arising in Severely Photodamaged Skin: A Case Report.

Cureus
2026

Radiotherapy-induced pemphigus vulgaris: a challenging case of extensive mucocutaneous ulcerations and literature review.

Oral surgery, oral medicine, oral pathology and oral radiology
2025

Clinical and Immunological Outcomes of Rituximab Therapy in Pemphigus: A Prospective Observational Study.

Dermatology (Basel, Switzerland)
2025

Cytomorphological Spectrum and Diagnostic Utility of Tzanck Smear Cytology in Vesiculobullous Skin Lesions: A Cross-Sectional Study From a Tertiary Care Center in Eastern Uttar Pradesh, India.

Cureus
2026

A systematic review of therapeutic and paradoxical roles of tumor necrosis factor α inhibitors in autoimmune blistering diseases.

Inflammopharmacology
2025

Rituximab-Based Management of Autoimmune Blistering Diseases: A Case Series Highlighting Ocular and Systemic Presentations.

Cureus
2026

A recalcitrant case of pemphigus treated with inebilizumab.

JAAD case reports
2025

Direct Immunofluorescence in Immunobullous Disorders of Skin With Histopathological Correlation Among Patients Attending a Tertiary Care Center.

Cureus
2025

Strongyloides stercoralis Hyperinfection in Pemphigus Vulgaris: A Neglected Preventable Fatality.

Acta medica portuguesa
2025

Pemphigus Vulgaris Mimicking Dyshidrotic Eczema: A Case Report.

Case reports in dermatology
2025

Real-world outcomes with rituximab vs. conventional therapy in pemphigus vulgaris: a single-center Romanian cohort.

Frontiers in medicine
2025

Sex-Specific Autoimmune Comorbidity Patterns in Pemphigus Vulgaris and Bullous Pemphigoid: A Bicenter Retrospective Case-Control Study.

Medicina (Kaunas, Lithuania)
2025

Patient-Reported Outcomes on Quality of Life in Older Adults with Oral Pemphigus.

Healthcare (Basel, Switzerland)
2026

A Rare Case of Pemphigus Vulgaris With Esophageal Involvement: Spontaneous Expulsion of a 50-cm Mucosal Lumenoid Fragment.

The American Journal of dermatopathology
2025

Desquamative Gingivitis and the Oral Microbiome: Insights into Immune-Microbial Interactions in Mucosal Inflammation.

Dentistry journal
2025

Updates in Pathogenesis of Pemphigus Vulgaris.

The Journal of dermatology
2026

Ultralow Dose Rituximab Combined With IVIG Is Safe and Effective in Pemphigus Vulgaris: Single-Center Review of 24 Patients.

The Australasian journal of dermatology
2025

[The role of KLF5 in cell adhesion and novel therapeutic approaches for pemphigus vulgaris].

Medecine sciences : M/S
2025

Successful Treatment of Refractory Severe Pemphigus With Telitacicept as Adjunctive Therapy: Two Case Reports.

International journal of dermatology
2026

Role of protein kinase C subtype-specific signalling in regulating adhesion in human keratinocytes and skin in pemphigus.

The British journal of dermatology
2025

Association of dietary diversity score and severity of pemphigus vulgaris: a cross-sectional study.

BMC nutrition
2025

Efficient On-Column Removal of Endotoxin from Immunoglobulins Such as AK23.

Current protocols
2026

Tumour necrosis factor-like weak inducer of apoptosis (TWEAK) is associated with inflammation and disease severity in pemphigus.

The British journal of dermatology
2026

Epithelial Barrier Diseases Among Adult Patients With Seborrheic Dermatitis.

JAMA dermatology
2025

Apremilast improves cardiomyocyte cohesion and arrhythmia in different models for arrhythmogenic cardiomyopathy.

Stem cell research &amp; therapy
2026

Boom or Bust: Clinical Trials in Pemphigus and Other B-Cell-Mediated Autoimmune Diseases.

The Journal of investigative dermatology
2025

A rare case report: retroperitoneal dedifferentiated liposarcoma associated with paraneoplastic pemphigus.

Frontiers in surgery
2025

Apremilast for Oral Mucosal-Predominant Resistant Pemphigus Vulgaris: A Promising Adjunctive Treatment.

Oral diseases
2025

Bead aggregation assays with desmoglein and desmocollin for the evaluation of disease activity in pemphigus.

Journal of dermatological science
2025

Coexistence of Psoriasis and Pemphigus: A Case Report of Pemphigus Vulgaris With a Review of the Literature.

Clinical case reports
2025

Misleading Hepatitis B Serology Following Intravenous Immunoglobulin Treatment for Pemphigus Vulgaris.

The Australasian journal of dermatology
2025

Eight Years of Follow-Up of Rituximab in Pemphigus Vulgaris and Foliaceus at a Single Center: Assessing Efficacy and Safety in Light of Several Factors.

Journal of clinical medicine
2025

The association between aromatic amino acids consumption and the severity of pemphigus vulgaris: a cross-sectional study.

Scientific reports
2025

Ofatumumab for the management of refractory pemphigus vulgaris: A case series.

JAAD international
2025

Crosstalk Between Keratinocytes and T Cells in Ulcerative Oral Mucosal Diseases: Mechanisms of Epithelial Dysfunction and Therapeutic Perspectives.

Journal of inflammation research
2025

Conversion of pathogenic T cells into functionally stabilized Treg cells for antigen-specific immunosuppression in pemphigus vulgaris.

Science translational medicine
2026

Biologic therapies for dermatologic emergencies: A comprehensive review.

Journal of the American Academy of Dermatology
2026

Esophageal Severe Manifestation of Pemphigus Vulgaris.

Digestive diseases and sciences
2025

Natural autoantibodies and their functional therapeutic roles in intravenous immunoglobulin.

Frontiers in aging
2025

[Pemphigus vulgaris - a case report].

Tierarztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
2025

Introducing human 3D skin models as a new serological diagnostic tool for severe autoimmune bullous diseases.

Frontiers in immunology
2025

Questionnaire Survey on Enterococcus faecalis-2001 Supplementation in 137 Patients With Oral Diseases at Two Facilities.

Cureus
2026

Pemphigus Vulgaris Scoring Systems: A Scoping Review.

Journal of oral pathology &amp; medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology
2025

Desquamative Gingivitis: Medical and Surgical Management of Oral Lesions.

Compendium of continuing education in dentistry (Jamesburg, N.J. : 1995)
2025

Taking aim at autoimmunity-Antigen-Fc constructs for mucosal pemphigus hit the mark.

Science immunology
2025

SERPINB5/TGF-β signaling modulates desmoplakin membrane localization and ameliorates pemphigus vulgaris skin blistering.

JCI insight
2026

Comparing combination and single-agent therapy for the treatment of pemphigus: A retrospective cohort study.

Journal of the American Academy of Dermatology
2025

Mucous membrane pemphigoid with ocular involvement in a patient diagnosed previously with mucosal-dominant pemphigus vulgaris: A case report.

JAAD case reports
2026

Deep Analysis of Desmoglein-3-Reactive CD4+ T Cells in Pemphigus Vulgaris Reveals a Type 17/Type 2 T-Cell Signature.

The Journal of investigative dermatology
2025

Are the dermatological cut-off values of commercially available ELISAs for BP180-NC16A appropriate when applied for diagnosis of oral mucous membrane pemphigoid?. Results of a preliminary study.

Minerva dental and oral science
2025

Clinical Insights of Kaposi's Varicelliform Eruption Associated with Underlying Primary Dermatoses- A Case Series from Hadoti Region of Rajasthan.

Indian journal of dermatology
2025

Species identification and antifungal susceptibility pattern of oral Candida isolates in pemphigus vulgaris: results from a case-control study.

Indian journal of medical microbiology
2025

Herpes Simplex Virus Type-1 Infection as a Trigger for Pemphigus Vulgaris.

Cureus
2025

Antigen-Fc fusion therapy reduces severity of a model of pemphigus vulgaris without systemic immunosuppression.

Science translational medicine
2025

Tofacitinib as a Novel Therapeutic Option for Pemphigus Vulgaris: A Case Report and Mechanistic Insights.

Clinical, cosmetic and investigational dermatology
2025

Desmoglein-driven dynamic signaling in pemphigus vulgaris: a systematic review of pathogenic pathways.

NPJ Regenerative medicine
2026

Immunoregulatory effects of TPM203 peptide-nanoparticle therapy in pemphigus vulgaris.

The British journal of dermatology
2025

Grinspan Syndrome Associated With Pemphigus Vulgaris Induced by COVID-19 Vaccination.

Cureus
2025

Longitudinal trends in immunological biomarkers, and predictors of relapse in pemphigus treated with rituximab: A prospective cohort study.

Indian journal of dermatology, venereology and leprology
2025

B cell-targeted therapies in autoimmune skin disease: current advances and challenges.

Current opinion in immunology
2025

Mitochondrial-dependent apoptosis and STAT3 activation induced by oxidative stress in pemphigus vulgaris.

Scientific reports
2025

Through the Microscope Lens: Investigating the Mechanisms of Desmosome Disruption in Pemphigus.

The Journal of investigative dermatology
2026

Safety, tolerability, pharmacokinetics and pharmacodynamic effects of desmoglein 3 peptide-coupled tolerizing nanoparticles in pemphigus.

The British journal of dermatology
2025

Novel Serum Oxidative Stress Biomarkers in Pemphigus Vulgaris: Clinical Insights and Implications for Pathogenesis.

Dermatology practical &amp; conceptual
2025

Endoscopic Findings of Mucosal Pemphigus Vulgaris: Clinical Correlation and Diagnostic Value.

Dermatology practical &amp; conceptual
2025

The effectiveness and safety of ofatumumab for the treatment of pemphigus vulgaris: a cohort study based on a registry database.

Frontiers in immunology
2025

Penile erosions: an atypical initial manifestation of pemphigus vulgaris.

Anais brasileiros de dermatologia
2025

Nail Involvement in Pemphigus: A Clinical and Dermoscopic Prospective Study.

Skin appendage disorders
2025

Data-driven image analysis to determine antibody-induced dissociation of cell-cell adhesion and antibody pathogenicity in pemphigus.

PNAS nexus
2025

Desmosomal cadherin tension loss in pemphigus vulgaris mediated by the inhibition of active RhoA at cell-cell adhesions.

iScience
2025

A Single Centre Cross-Sectional Observational Study on Dermatoscopy of Oral Mucosal Disorders and Histopathological Correlation in Tertiary Care Centre from Central India.

Indian journal of dermatology
2025

Dupilumab as Immunomodulatory Rescue for Severe Recalcitrant Pemphigus Vulgaris: A Case Report and Literature Review.

Clinical, cosmetic and investigational dermatology
2025

A Clinico-Epidemiological Study of Various Autoimmune Vesiculobullous Disorders in a Tertiary Care Center.

Cureus
2025

Rituximab-induced subcutaneous sarcoidosis in a patient with refractory pemphigus vulgaris.

Therapie
2025

Brown patches and erosions with an erythematous base.

JAAD case reports
2025

Ultra-Low-Dose Rituximab in Pemphigus Vulgaris: How Low is the Lowest?

Indian dermatology online journal
2025

Low Dose Vs Conventional Rituximab Regimen in Pemphigus Vulgaris - A Retrospective Single Centre Comparative Analysis to Assess the Clinical Efficacy and Cost-Effectiveness.

Indian dermatology online journal
2025

Assessment of Efficacy of Adjuvant Topical Rituximab Encapsulated in Nanoparticle Gel in Oral Pemphigus Vulgaris: A Randomized, Double-Blind, Placebo-Controlled, Pilot Study.

Indian dermatology online journal
2025

A Case Report of Pemphigus Vulgaris Initially Misdiagnosed as Tinea Capitis: Infectious Consequences of Diagnostic Delay in a Patient Treated With Rituximab.

Cureus
2025

Pulmonary cryptococcosis in the setting of immunosuppression by methylprednisolone monotherapy for oral pemphigus: a case report and literature review.

Frontiers in medicine
2025

Evaluation of total sulfhydryl levels and dietary habits in pemphigus vulgaris.

Postepy dermatologii i alergologii
2025

The Role of Microbiota in the Pathogenesis of Bullous Pemphigoid and Pemphigus Vulgaris: Evidence, Controversies, and Perspectives.

International journal of molecular sciences
2025

Correlation of Antimuscarinic Acetylcholine Receptor Antibody Titers With Pemphigus Disease Activity at Baseline and Following Phase I Pulse Therapy.

Cureus
2025

A 10-Year Observational Study on Treatment Approaches in Pemphigus and Pemphigoid.

Acta stomatologica Croatica
2025

Role of Desmoglein Autoantibody in the Diagnosis of Pemphigus Vulgaris: A Case Report.

Acta medica Indonesiana
2025

Pemphigus vulgaris following radiotherapy in head and neck cancer: Clinicopathological perspective of a rare cutaneous manifestation after radiotherapy treatment.

Journal of cancer research and therapeutics
2025

Treatment of pemphigus and other neglected skin conditions with PC111, a human anti-Fas Ligand monoclonal antibody: a potential disease modifier.

The Journal of dermatological treatment
2025

Unusual Presentation of Pemphigus Vulgaris as a Solitary Ulcerative Scalp Lesion: A Case Report.

Cureus
2025

Alterations in chemokine receptor-ligand interactions and costimulatory molecules in DC-NK crosstalk: A novel therapeutic approach for pemphigus vulgaris.

Immunology letters
2025

Nonfatal Strongyloides Stercoralis Hyperinfection Secondary to Pemphigus Vulgaris Immunosuppressant Treatment: Case Report and Brief Literature Review.

Acta medica portuguesa
2025

Rilzabrutinib-induced transition from pemphigus vulgaris to pemphigus foliaceous: a case report and literature review.

Dermatology reports
2025

Pulmonary Inflammatory Myofibroblastic Tumor in a Patient Treated for Pemphigus Vulgaris: A Case Report.

Cureus
2025

Circulating Antibodies Against DSG1 and DSG3 in Patients with Oral Lichen Planus: A Scoping Review.

Antibodies (Basel, Switzerland)
2025

HLA DRB1* Allele Lupus Erythematosus, Rheumatoid Arthritis, and other Autoimmune Disorders with Skin Involvement.

Mediterranean journal of rheumatology
2025

The role of B cell-activating factor system in autoimmune diseases: mechanisms, disease implications, and therapeutic advances.

Frontiers in immunology
2025

A Multimodal Approach to Diagnosis of Immunobullous Diseases: Integrating Clinical, Histopathological, and Immunofluorescence Findings.

Cureus
2025

Rare Ocular Association With Hailey-Hailey Disease.

Ochsner journal
2025

Treatment of refractory pemphigus vulgaris with efgartigimod.

JAAD case reports
2025

Pemphigus Vulgaris: A rare cause of esophageal bleeding and stricture.

Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
2025

The Efficacy of Dupilumab in Pemphigus Vulgaris.

International journal of dermatology
2025

Correlation and assessment of vitamin D deficiency with disease severity in patients suffering from pemphigus vulgaris.

JPMA. The Journal of the Pakistan Medical Association
2026

Pioneering Palliative Care in Pemphigus Vulgaris: A Landmark Case Report from Myanmar.

Journal of palliative care
2025

Antigen-specific immune responses and microbiota interactions in skin: Insights into autoimmune skin diseases and emerging therapeutic strategies.

The Journal of allergy and clinical immunology
2026

The Multikinase Inhibitor Midostaurin Mitigates Loss of Intercellular Adhesion and Skin Blistering in Pemphigus Vulgaris.

The Journal of investigative dermatology
2025

Evaluating the Role of Low-Dose Methotrexate in Pemphigus Vulgaris: A Commentary on Recent Findings.

The Journal of dermatology
2025

Factors associated with persistent anti-desmoglein positivity after remission in pemphigus vulgaris: a prospective registry-based cohort study.

Frontiers in immunology
2025

Juvenile pemphigus vulgaris: A narrative review.

Medicine
2025

Successful treatment of pemphigus vulgaris with obinutuzumab.

The British journal of dermatology
2025

Risk of Osteoporosis Associated with Glucocorticoid Use in Pemphigus Vulgaris: Insights from a Retrospective Cohort Study.

Dermatology practical &amp; conceptual
2025

Pemphigus Vulgaris: Clinical Aspects and Treatments.

Advances in skin &amp; wound care
2025

JAK1/2 Inhibitors Alleviate the Damage of Intercellular Adhesion by Reducing Endoplasmic Reticulum Stress-Induced Apoptosis in Pemphigus Vulgaris.

Experimental dermatology
2025

Efficacy, safety, and B-cell depletion capacity of 3 rituximab dosing regimens in the treatment of moderate-to-severe pemphigus vulgaris and pemphigus foliaceus: A 52-week clinical trial.

Journal of the American Academy of Dermatology
2025

High-Sensitivity Cell-Based Plasmonic Biolayer Interferometry with a Two-Tiered Enhancement for Clinical Autoimmunity Surveillance of Pemphigus Vulgaris.

ACS nano
2025

A red nodule in the umbilicus of an older man.

Skin health and disease
2025

Mucoscopic Features of Oral Lichen Planus: A Retrospective Comparative Study with Inflammatory Mimickers.

Diagnostics (Basel, Switzerland)
2025

Management of Oral Mucositis in Pediatric Patients: A Practical Overview of Available Treatment.

Pediatric dermatology
2025

Correlating pemphigus disease area index (PDAI) severity categories with oral disease severity score (ODSS) in oral mucosal pemphigus - A pilot study.

Indian journal of dermatology, venereology and leprology
2025

The application of direct immunofluorescence in the pathological diagnosis of oral mucosal diseases: Series of 116 cases.

Pathology, research and practice
2025

Epac1 contributes to apremilast-mediated rescue of pemphigus autoantibody-induced loss of keratinocyte adhesion.

JCI insight
2025

Elevated Fab glycosylation of autoantibodies maintained during B cell depletion therapy.

Scientific reports
2025

Severe psoriasis vulgaris complicating pemphigus vulgaris: A case report.

Medicine
2025

[CAR-T cells in Dermatology: Mechanisms of action and applications in autoimmune diseases].

Revista medica del Instituto Mexicano del Seguro Social
2025

JAK-STAT pathway, type I/II cytokines, and new potential therapeutic strategy for autoimmune bullous diseases: update on pemphigus vulgaris and bullous pemphigoid.

Frontiers in immunology
2025

Direct inhibition precedes p38 mitogen activated protein kinase-mediated uncoupling in desmosomes to reduce desmoglein 3 adhesion by pemphigus autoantibodies.

The British journal of dermatology
2025

The Liraglutide-Induced Pemphigus Vulgaris: A First Case Report Highlighting Autoimmune Risks of GLP-1 Receptor Agonists.

Endocrine, metabolic &amp; immune disorders drug targets
2025

Risk Factors Associated with Oral Candidiasis in Pemphigus Vulgaris Patients: Results from a Case-Control Study in India.

The American journal of tropical medicine and hygiene
2025

Genetic variations in the ST18 gene and their association with pemphigus vulgaris in Vietnamese patients: insights from a case series.

Dermatology reports
2025

Association of pemphigus vulgaris with infections in end-stage renal disease patients.

The American journal of the medical sciences
2025

Outcome measurement in pemphigus clinical research: a scoping review on heterogeneity in outcome reporting and definitions.

The British journal of dermatology
2025

Cutaneous epidermal growth factor receptor expression (EGFR) and soluble EGFR levels are elevated in patients with pemphigus vulgaris: a longitudinal study.

Archives of dermatological research
2025

Clinical outcomes and prognostic factors of pemphigus vulgaris and pemphigus foliaceus: A 20-year retrospective study.

Chinese medical journal
2025

A retrospective analysis of oral autoimmune bullous diseases at a Thai oral medicine center.

Journal of dental sciences
2025

Integration of Longitudinal Clinical, Immunologic, and Environmental Data for Enhanced Disease Monitoring and Management in Pemphigus Vulgaris: A Case Study.

JID innovations : skin science from molecules to population health
2025

Interleukin (IL)-34 promotes the inflammatory role of IL-1β-producing myeloid cells in pemphigus lesions.

The British journal of dermatology
2025

Rare Presentation of Rituximab-induced Interstitial Lung Disease in a Patient with Pemphigus Vulgaris.

The Journal of the Association of Physicians of India
2025

Plasmapheresis for Facilitating Readministration of Rituximab After Paradoxical Exacerbation in Pemphigus Vulgaris: A Case Report.

Clinical case reports
2025

MicroRNAs in patients with pemphigus: A systematic review.

International immunopharmacology
2025

Trichoscopy of Pemphigus Vulgaris and Foliaceus on Black Scalp.

Skin appendage disorders
2025

Rare Coexistence of Pemphigus Vulgaris and Eosinophilic Esophagitis: A Report of Two Cases.

Clinical case reports
2025

Lipid Profile Scores Predict Severity of Pemphigus: A Cross-Sectional Study.

Indian journal of dermatology
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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Iatrogenic Upper Gastrointestinal Bleeding Following Nasogastric Tube Insertion in a Patient with Pemphigus Vulgaris.
    The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology· 2026· PMID 41846486mais citado
  2. Caspase-activation powers anti-Desmoglein 3-induced acantholysis in human epidermis.
    Cell death discovery· 2026· PMID 41714593mais citado
  3. [Value of direct immunofluorescence in the diagnosis of oral mucosal pemphigus vulgaris: A retrospective study based on multi-index combined analysis].
    Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences· 2026· PMID 41633587mais citado
  4. 3D organotypic skin models recapitulate autoantibody-driven pemphigus pathomechanisms and targeted therapeutic response.
    Science advances· 2026· PMID 41512055mais citado
  5. Epithelial Barrier Diseases Among Adult Patients With Seborrheic Dermatitis.
    JAMA dermatology· 2026· PMID 41191376mais citado
  6. Nature's Bandage: A Memorable Rescue of Severe Pemphigus Vulgaris in a Resource-Limited Setting.
    Clin Exp Dermatol· 2026· PMID 41986291recente
  7. Kaposi Sarcoma Associated with Pemphigus Vulgaris in the Setting of Immunosuppressive Therapy: Case Report and Review of Literature.
    Case Rep Dermatol· 2026· PMID 41983146recente
  8. Critical analysis of pemphigus vulgaris in pregnancy.
    Front Immunol· 2026· PMID 41983144recente
  9. Single amino acid substitutions modulate the function of α9α10 nicotinic acetylcholine receptor.
    Int J Biol Macromol· 2026· PMID 41966377recente
  10. Lesion visibility and localization as predictors of social appearance anxiety in Pemphigus vulgaris: a case-control study.
    Psychol Health Med· 2026· PMID 41962002recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:704(Orphanet)
  2. OMIM OMIM:169610(OMIM)
  3. MONDO:0008219(MONDO)
  4. GARD:7355(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q3899001(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Pênfigo vulgar
Compêndio · Raras BR

Pênfigo vulgar

ORPHA:704 · MONDO:0008219
Prevalência
1-5 / 10 000
Herança
Not applicable
CID-10
L10.0 · Pênfigo vulgar
CID-11
Ensaios
8 ativos
Início
Adult, Childhood, Elderly
Prevalência
18.0 (Europe)
MedGen
UMLS
C0030809
EuropePMC
Wikidata
Papers 10a
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