A adesão celular é o processo pelo qual as células interagem e se ligam às células vizinhas por meio de moléculas especializadas da superfície celular. Esse processo pode ocorrer por meio do contato direto entre as superfícies das células, como as junções celulares, ou por meio da interação indireta, em que as células se ligam à matriz extracelular circundante, uma estrutura semelhante a um gel que contém moléculas liberadas pelas células nos espaços entre elas. A adesão celular ocorre a partir das interações entre as moléculas de adesão celular (CAMs), proteínas transmembrana localizadas na superfície celular. A adesão celular liga as células de diferentes maneiras e pode estar envolvida na transdução de sinais para que as células detectem e respondam a mudanças no ambiente.
Introdução
O que você precisa saber de cara
Pênfigo IgA é uma doença autoimune rara caracterizada por elevação monoclonal de IgA circulante e infiltração neutrofílica da pele. Manifesta-se com bolhas na mucosa oral, pústulas, erosões e lesões cutâneas anulares, vesículas e placas, podendo haver eosinofilia.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 19 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Pênfigo IgA
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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
Ensaios em destaque
Pesquisa e ensaios clínicos
1 ensaios clínicos encontrados.
Publicações mais relevantes
A 14-year multicentric follow-up study of atypical pemphigus variants in Italy: the VARIANT_P study.
The clinical, epidemiological and immunopathological profiles of atypical forms of pemphigus remain only partially known. To define the clinical, epidemiological and immunological characteristics, therapies and outcomes in patients with atypical pemphigus variants. This was a 14-year multicentre retrospective observational study (VARIANT_P) on atypical variants of pemphigus across Italy. We collected demographic, immunopathological and clinical data, as well as information on comorbidities and prescribed treatments. We enrolled 61 patients [female/male sex ratio 1.77; 13 paraneoplastic pemphigus (PNP), 26 IgA pemphigus (PIgA), 22 pemphigus herpetiformis (PH)]. The median ages at onset and diagnosis were 70.6 (range 43.1-86.8) and 71.1 (range 46.9-86.9) for PNP; 62.2 (range 3.8-81.0) and 63.6 (range 4.0-82.4) for PIgA; and 49.4 (range 5.4-84.4) and 52.3 (range 5.9-85.9) for PH, respectively. The median diagnostic delay was 3.0 (range 0.0-45.6) months for PNP, 9.5 (range 1.0-140.0) months for PIgA and 2.0 (range 0-30.4) months for PH. The mortality rate was 55% (6/11) for PNP, 4% (1/26) for PIgA and 6% (1/17) for PH. Cutaneous involvement was present in all patients with PIgA and PH, and in 83% (10/12) of the patients with PNP. In contrast, oral mucosal involvement was observed in all patients with PNP with data (n = 12), but only in 8% (2/26) of those with PIgA and 21% (4/19) of those with PH. Histology, direct immunofluorescence, indirect immunofluorescence and enzyme-linked immunosorbent assay data demonstrated variable concordance with previously known data. Comorbidities included mainly solid malignancies for people with PNP, whereas cardiovascular and metabolic diseases were the most prevalent for those with PIgA and PH. Treatment mostly relied on systemic steroids and rituximab. The VARIANT_P study contributes to data collection relating to atypical pemphigus variants in order to promote the development of specific therapeutical guidelines in the future.
[Divided bullae: half-half blisters in IgA pemphigus].
Generalized Annular Pustular Lesions in a Child.
IgA pemphigus following SARS-Cov-2 vaccine.
Treatment Outcomes in IgA Pemphigus: A Systematic Review.
Publicações recentes
A 14-year multicentric follow-up study of atypical pemphigus variants in Italy: the VARIANT_P study.
[Divided bullae: half-half blisters in IgA pemphigus].
Generalized Annular Pustular Lesions in a Child.
IgA pemphigus following SARS-Cov-2 vaccine.
Treatment Outcomes in IgA Pemphigus: A Systematic Review.
📚 EuropePMC107 artigos no totalmostrando 68
A 14-year multicentric follow-up study of atypical pemphigus variants in Italy: the VARIANT_P study.
Clinical and experimental dermatology[Divided bullae: half-half blisters in IgA pemphigus].
Dermatologie (Heidelberg, Germany)Generalized Annular Pustular Lesions in a Child.
Pediatric dermatologyIgA pemphigus following SARS-Cov-2 vaccine.
Italian journal of dermatology and venereologyTreatment Outcomes in IgA Pemphigus: A Systematic Review.
Journal of cutaneous medicine and surgeryArciform Erythematous Plaques With Hypopyon Sign.
International journal of dermatologyUncommon and Unusual Variants of Autoimmune Bullous Diseases.
Indian dermatology online journalA case of IgA pemphigus, with a poor response to dapsone, successfully treated with adalimumab.
Clinical case reportsIgA pemphigus as an immune checkpoint inhibitor-associated skin manifestation.
JAAD case reportsA case of IgA pemphigus with acantholysis in oral mucosal lesions.
Skin health and diseaseSuperficial and Bullous Neutrophilic Dermatoses: Sneddon-Wilkinson, IgA Pemphigus, and Bullous Lupus.
Dermatologic clinicsNeutrophilic and eosinophilic dermatoses associated with hematological malignancy.
Frontiers in medicine[Refraktärer IgA-Pemphigus erfolgreich mit Adalimumab als Monotherapie behandelt].
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGRefractory IgA pemphigus successfully managed with adalimumab as monotherapy.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGRefractory atypical IgA pemphigus successfully treated with apremilast.
The Journal of dermatologyIgA pemphigus following COVID-19 vaccination: A case report.
SAGE open medical case reportsSuccessful treatment with narrowband UVB in a recalcitrant case of IgA pemphigus.
Anais brasileiros de dermatologiaPersistent Scattered Pustules: Vesiculopustular IgA Pemphigus Arising in a Patient With Ulcerative Colitis.
The American Journal of dermatopathologyDermoscopic approach for differential diagnosing of autoimmune bullous disease: pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus.
International journal of dermatologyCase report: Vesiculobullous eruption with an atypical IgA deposition pattern in a patient with multiple myeloma-A case report and literature review.
Frontiers in immunologyIgA pemphigus and Sneddon Wilkinson disease: a spectrum of diseases?
Italian journal of dermatology and venereologyIgG/IgA pemphigus with differing regional presentations.
JAAD case reportsClinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study.
Frontiers in immunologyVintage descriptions of IgA pemphigus.
Journal of the European Academy of Dermatology and Venereology : JEADVSubcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma: A case report and literature review.
The Journal of dermatologyCurrent and Innovated Managements for Autoimmune Bullous Skin Disorders: An Overview.
Journal of clinical medicineClinical, Histopathologic, and Immunohistochemical Features of Patients with IgG/IgA Pemphigus.
BiomedicinesUtility of IgG4 immunohistochemistry detection in pemphigus diagnosis.
SAGE open medical case reportsEpidemiology of Pemphigus.
JID innovations : skin science from molecules to population healthAn Updated Review of Pemphigus Diseases.
Medicina (Kaunas, Lithuania)Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review).
Experimental and therapeutic medicineThe epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016.
PloS onePathogenic Activation and Therapeutic Blockage of FcαR-Expressing Polymorphonuclear Leukocytes in IgA Pemphigus.
The Journal of investigative dermatologyA Case of Dapsone-induced Mild Methemoglobinemia with Dyspnea and Cyanosis.
Acta dermatovenerologica Croatica : ADCIntraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.
Journal of the American Academy of DermatologyImmune serological diagnosis of pemphigus.
Italian journal of dermatology and venereologyA retrospective study on the clinicopathologic features of IgG/IgA pemphigus.
Journal of the American Academy of DermatologyIgA pemphigus and linear IgA bullous dermatosis in a patient with ulcerative colitis.
The Australasian journal of dermatologyA case of tinea incognito mimicking subcorneal pustular dermatosis.
Dermatology online journalIgA pemphigus: A systematic review.
Journal of the American Academy of DermatologyIgG/IgA pemphigus in a patient with a history of pemphigus vulgaris: An example of epitope spreading?
Journal of cutaneous pathologyImatinib-induced IgA Pemphigus: Subcorneal Pustular Dermatosis Type.
Indian dermatology online journalIgG/IgA pemphigus: Report of a rare subtype of pemphigus.
The Australasian journal of dermatologySystemic retinoids for treatment of recalcitrant IgA pemphigus.
Orphanet journal of rare diseasesClinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis.
Frontiers in immunologyPemphigus group: overview, epidemiology, mortality, and comorbidities.
Immunologic researchA case of IgA pemphigus foliaceus with high scores on drug-induced lymphocyte stimulation tests secondary to various drugs.
European journal of dermatology : EJDAnti-desmoglein 1 IgG/IgA pemphigus associated with thymoma.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGIntraepidermal neutrophilic dermatosis type of IgA pemphigus with circulating linear IgA disease antibodies associated with ulcerative colitis.
Journal of the European Academy of Dermatology and Venereology : JEADVIgA pemphigus: lumping or splitting?
The British journal of dermatologyUtility of immunofluorescence in dermatology.
Indian dermatology online journalDiscrepancies among clinical, histological and immunological findings in IgA pemphigus: a case report and literature survey.
Postepy dermatologii i alergologiiCrusted impetigo-like lesion on the face: a case of IgG/IgA pemphigus.
Journal of the European Academy of Dermatology and Venereology : JEADVIgA pemphigus showing IgA antibodies to desmoglein 1 and 3.
Dermatology practical & conceptualGeneralized pustular psoriasis induced by systemic steroid dose reduction.
Anais brasileiros de dermatologiaComplete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with antimyeloma therapy: association with disappearance of M-protein.
The British journal of dermatologyBullous Pemphigoid with Lymphocytic Colitis: A Case Report and Short Literature Review.
Dermatology and therapySubcorneal Pustular Dermatosis: A Review of 30 Years of Progress.
American journal of clinical dermatologyA Clinicopathological Study of Pemphigus in Eastern India with Special Reference to Direct Immunofluorescence.
Indian journal of dermatologyJuvenile IgA pemphigus: A case report and review of literature.
Indian journal of dermatology, venereology and leprologyClinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at Kurume University.
The British journal of dermatologyProlonged pustular eruption from hydroxychloroquine: an unusual case of acute generalized exanthematous pustulosis.
CutisChildhood Pemphigus Foliaceus with Exclusive Immunoglobulin G Autoantibodies to Desmocollins.
Pediatric dermatologyClinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus.
International journal of dermatologyCase of pemphigus with immunoglobulin G and A antibodies, binding to both the intercellular spaces and basement membrane zone.
The Journal of dermatologyImmunobullous disease and ulcerative colitis: a case series of six patients.
The British journal of dermatologyIgG/IgA pemphigus representing pemphigus vegetans caused by low titres of IgG and IgA antibodies to desmoglein 3 and IgA antibodies to desmocollin 3.
Journal of the European Academy of Dermatology and Venereology : JEADVSuccessful treatment of Wilson disease-associated IgA pemphigus with IVIG.
Journal of the European Academy of Dermatology and Venereology : JEADVAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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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.
- A 14-year multicentric follow-up study of atypical pemphigus variants in Italy: the VARIANT_P study.
- [Divided bullae: half-half blisters in IgA pemphigus].
- Generalized Annular Pustular Lesions in a Child.
- IgA pemphigus following SARS-Cov-2 vaccine.
- Treatment Outcomes in IgA Pemphigus: A Systematic Review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:555905(Orphanet)
- MONDO:0034127(MONDO)
- GARD:22242(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
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
