Necrólise epidérmica tóxica (NET) ou Síndrome de Lyell é um tipo de reação adversa cutânea grave que afeta mais de 30% da pele. Começa com sintomas de gripe e alguns dias depois, aparecem vesículas e descamação dolorosa em áreas da pele ou das membranas mucosas, como a boca. As complicações incluem a desidratação, sepse, pneumonia e falência de múltiplos órgãos. A síndrome de Stevens–Johnson (SJS) é muito similar, mas afeta menos de 10% da pele. Uma forma intermédia NET/SJS afeta entre 10 e 30% da pele.
Introdução
O que você precisa saber de cara
Dermatose tóxica é uma doença rara associada a genes como HLA-B e IKZF1, manifestando-se com bolhas genitais, edema facial, secura vaginal e uveíte anterior. Pode apresentar calafrios, simbléfaro, vulvodinia e alterações na faringe, com leucocitose associada.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 51 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 136 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
Genes associados
3 genes identificados com associação a esta condição.
Antigen-presenting major histocompatibility complex class I (MHCI) molecule. In complex with B2M/beta 2 microglobulin displays primarily viral and tumor-derived peptides on antigen-presenting cells for recognition by alpha-beta T cell receptor (TCR) on HLA-B-restricted CD8-positive T cells, guiding antigen-specific T cell immune response to eliminate infected or transformed cells (PubMed:23209413, PubMed:25808313, PubMed:29531227, PubMed:9620674). May also present self-peptides derived from the
Cell membraneEndoplasmic reticulum membrane
Stevens-Johnson syndrome
A rare blistering mucocutaneous disease that share clinical and histopathologic features with toxic epidermal necrolysis. Both disorders are characterized by high fever, malaise, and a rapidly developing blistering exanthema of macules and target-like lesions accompanied by mucosal involvement. Stevens-Johnson syndrome is a milder disease characterized by destruction and detachment of the skin epithelium and mucous membranes involving less than 10% of the body surface area. Ocular symptoms include ulcerative conjunctivitis, keratitis, iritis, uveitis and sometimes blindness. It can be caused by a severe adverse reaction to particular types of medication, although Mycoplasma infections may induce some cases.
Transcription regulator of hematopoietic cell differentiation (PubMed:17934067). Binds gamma-satellite DNA (PubMed:17135265, PubMed:19141594). Plays a role in the development of lymphocytes, B- and T-cells. Binds and activates the enhancer (delta-A element) of the CD3-delta gene. Repressor of the TDT (fikzfterminal deoxynucleotidyltransferase) gene during thymocyte differentiation. Regulates transcription through association with both HDAC-dependent and HDAC-independent complexes. Targets the 2
NucleusCytoplasm
Antigen-presenting major histocompatibility complex class I (MHCI) molecule. In complex with B2M/beta 2 microglobulin displays primarily viral and tumor-derived peptides on antigen-presenting cells for recognition by alpha-beta T cell receptor (TCR) on HLA-A-restricted CD8-positive T cells, guiding antigen-specific T cell immune response to eliminate infected or transformed cells (PubMed:10449296, PubMed:12138174, PubMed:12393434, PubMed:1402688, PubMed:15893615, PubMed:17189421, PubMed:19543285
Cell membraneEndoplasmic reticulum membrane
Medicamentos e terapias
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: TNF-alpha inhibitor
Mecanismo: Cyclophilin A modulator
Mecanismo: TNF-alpha inhibitor
Mecanismo: Fibroblast growth factor receptor 2 agonist
Mecanismo: Janus Kinase (JAK) inhibitor
Mecanismo: Tyrosine-protein kinase JAK1 inhibitor
Variantes genéticas (ClinVar)
108 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
12 vias biológicas associadas aos genes desta condição.
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 — Dermatose tóxica
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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
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Breast (Edinburgh, Scotland)Integrative transcriptomic and machine learning framework reveals candidate genes and potential mechanisms of aflatoxin B1 exposure in breast cancer.
Scientific reportsProton therapy in breast cancer: bridging medical and radiation oncology.
Cancer treatment reviewsHigh-throughput engineered tumor organoids reveal ROCK signaling as an immunotherapeutic target in triple-negative breast cancer.
Cell reportsRemote ischemic conditioning protects against anthracycline cardiotoxicity without impairing its antitumor activity.
Basic research in cardiologyDiscovery of Novel 1,3,5-trisubstituted-2-thioxo-imidazole Derivatives as Potential Aromatase Inhibitors: Design, Synthesis, In Vitro Anti-Breast Cancer Screening and Molecular Docking Studies.
Drug development researchBeyond Chemotherapy: Network Meta-Analysis Reveals Optimal Neoadjuvant Strategies for Luminal Breast Cancer.
Cancer medicineThe Complement System in ANCA-Associated Vasculitis: Mechanistic Insights, Therapeutic Horizons, and Unmet Clinical Needs.
Immunity, inflammation and diseasePluronic® F127 Polymeric Micelles as Nanocarriers for Pentamidine: Improving Safety and Biological Efficacy Against Leishmania major.
International journal of molecular sciencesUHPLC-QTOF-MS Profiling of Chemical Constituents in POW9TM Cocktail with Antioxidant and Anti-Proliferative Potentials Against Vero, MCF-7 and MDA-MB-231 Cells.
International journal of molecular sciencesAX-2: A Promising Non-Hemolytic Protein of Bacillus thuringiensis with Potent Selective Cytotoxicity Against Breast Cancer Cells.
Molecules (Basel, Switzerland)Microparticles against leishmaniasis: Advances, challenges, and perspectives in modified drug delivery systems.
Microbial pathogenesisStructural reprogramming of irradiated ellagic acid suppresses cell cycle regulators and modulates cytokines via activation of the p53/BAX-CASP3/Bcl-2-cyclin D1 pathway: Validation studies across breast cancer cell lines.
International journal of biological macromoleculesEuropean S2k guidelines on management of autoimmune blistering diseases in children and adolescents.
Journal of the European Academy of Dermatology and Venereology : JEADVRemoval of volatile organic compounds by chemical filters significantly inhibited the development of atopic dermatitis symptoms in mice: potential implications for air-conditioning systems in healthcare environments.
Toxicological sciences : an official journal of the Society of ToxicologyFirst-line ipilimumab plus nivolumab in advanced merkel cell carcinoma: a meta-analysis of prospective trials and real-world validation cohort.
Cancer immunology, immunotherapy : CIICisplatin(IV)-fatty acid prodrug-bound albumin nanoparticles: In vitro, in silico, and in vivo evaluation in breast cancer therapy.
Nanomedicine : nanotechnology, biology, and medicinePrevention and management of amivantamab-induced dermatologic toxicities: a European expert consensus and practical algorithm.
Lung cancer (Amsterdam, Netherlands)Molecular docking approaches in mycetoma: Toward improved patient management.
PLoS neglected tropical diseasesAn in situ-forming injectable silk hydrogel with a cocktail of chemotherapeutic drugs simultaneously targeting cancer cells and cancer stem cells.
Journal of materials chemistry. BBiometallic peptide-drug conjugates in photo-crosslinkable hydrogels enable combined photothermal-chemotherapy against breast cancer.
Journal of nanobiotechnologyInjectable hydrogel induces tumor cell extracellular calcification and bone regeneration to disrupt the osteolytic vicious cycle in bone metastasis.
Journal of controlled release : official journal of the Controlled Release SocietySuccessful Management of Progressive Mycetoma Affecting the Head and Neck.
The American journal of tropical medicine and hygieneUltrasmall Platinum Nanoclusters Modulating Dysregulated Reactive Oxide Species and Immunity for Psoriasis Therapy and Prevention.
ACS nanoGreen Synthesized Silver Nanoformulation Using Rhubarb Extract for Antibacterial and Anticancer Therapy in Infection-Associated Breast Cancer Mice.
ACS applied materials & interfacesTamoxifen-loaded TPGS-PLGA nanoplatform for breast cancer therapy: insights into drug delivery, distribution, treatment efficacy, and ultrasound/photoacoustic imaging.
Nanomedicine (London, England)Toxic epidermal necrolysis associated with immune checkpoint inhibitors for bladder cancer: A case report.
Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences[Data mining and toxicity profile analysis of immune checkpoint inhibitor-related skin toxicity events based on FAERS].
Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciencesFrom Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events.
Seminars in arthritis and rheumatismHierarchical pluronic F127-chitosan hydrogel embedding gallium-doxorubicin co-loaded nanocapsules enables dual-responsive release and apoptosis in triple-negative breast cancer cells.
Colloids and surfaces. B, BiointerfacesTargeted Therapy for CD13-Overexpressing Triple-Negative Breast Cancers Using Apoptosis-Inducing Protein Cage Nanoparticles.
Macromolecular bioscienceIntegrating antibody-drug conjugates into the management of early breast cancer.
Cancer treatment reviewsClassification of dermal sensitizers under the globally harmonized system of classification and labelling of chemicals (GHS): A critical review of the principles and current challenges.
Toxicology and industrial healthTriple negative breast cancer: what clinical progress have we seen in the last 5 years?
Expert opinion on investigational drugsEfficacy of high doses of ivermectin-clorsulon in cattle on Glossina palpalis gambiensis survival and fecundity: implications for human and animal trypanosomoses control.
Parasites & vectorsAfuresertib plus fulvestrant for pretreated HR-positive, HER2-negative, advanced breast cancer: a phase Ib trial.
Nature communicationsResminostat for maintenance treatment in patients with advanced-stage mycosis fungoides or Sézary syndrome: a multicentre, double-blind, randomised, placebo-controlled, phase 2 trial.
The Lancet. HaematologyNanoparticle-loaded microneedles as advanced transdermal systems for skin cancer management.
International journal of pharmaceuticsMagnetic hyperthermia's potential in triple-negative breast cancer treatment.
Progress in biophysics and molecular biologyDevelopment of anti-EGFR targeted magnetic nanoparticles for doxorubicin delivery into triple negative breast cancer cells.
European journal of pharmaceutics and biopharmaceutics : official journal of Arbeitsgemeinschaft fur Pharmazeutische Verfahrenstechnik e.VMechanistic insights into sitagliptin-induced skin autoimmunity: Linking environmental drug exposure to IL-6 activation in bullous pemphigoid.
Ecotoxicology and environmental safetyGlycolysis-dependent sulfur metabolism orchestrates morphological plasticity and virulence in fungi.
eLifeTargeting Hypoxic Tumor Microenvironments: Biocompatible and Stable MPC-BA Micelles in Breast Cancer Treatment.
ACS applied bio materialsToward Microbiome-Informed Melanoma Care: The Gut Microbiota in Melanoma Evolution, Immunotherapy Response and Immune-Related Toxicity.
Current oncology reportsTargeted Neutrophil Extracellular Traps Mimics Combat Staphylococcus aureus Infections.
ACS infectious diseasesAssociations of prenatal exposure to organophosphate pesticides with eczema and allergic rhinoconjunctivitis in 4-year-old children: The Japan Environment and Children's Study.
Environmental researchHidden risks associated with occupational pesticide exposure in women with breast cancer: High frequency of the Luminal B molecular subtype and occurrence of poor prognostic features.
PloS oneExosome-based strategies and immunotherapy for skin cancer: mechanisms, challenges, and future directions.
Medical oncology (Northwood, London, England)A multidisciplinary, phased nursing strategy for skin and mucosal management in a pediatric case of toxic epidermal necrolysis with respiratory failure: a case report.
Frontiers in pediatricsRaspberry protective role in inflammatory diseases: An overview.
Iranian journal of basic medical sciencesSurvival and toxicity profile of elderly melanoma patients treated with immune checkpoint inhibitors: a retrospective study from a tertiary care center.
Melanoma researchDerivation of a Point of Departure using NAMs for application in Quantitative Risk Assessment of fragrance materials.
Regulatory toxicology and pharmacology : RTPAn insight into the role of nanotechnology in photothermal and photodynamic therapy of leishmaniasis.
Drug discovery todayCustomized Non-invasive Brachytherapy With Tailored Surface Moulds: An Experience From a Tertiary Care Centre.
Clinical oncology (Royal College of Radiologists (Great Britain))Cyclic di-GMP suppresses cancer metastasis by targeting proteasome 26S subunit non-ATPase 3 independently of STING.
Signal transduction and targeted therapyAssociações
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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.
- Autologous stem cell transplant for severe, progressive juvenile systemic sclerosis.
- Antimicrobial Peptides: Structure, Mechanisms, and Therapeutic Potential in Combating Infectious Diseases.
- Erosive pustular dermatosis-like scalp reaction following cranial radiotherapy in a patient with EGFR-mutant NSCLC treated with amivantamab.
- Cancer Therapy-Related Cardiac Dysfunction in Breast Cancer Patients Receiving Combination Therapy of Candesartan and Carvedilol for Primary Prevention: A Prospective Longitudinal Study.
- Out of Nucleus: Serine 727 Phosphorylation Orchestrates Non-Canonical STAT3 Functions-Relevance to Triple-Negative Breast Cancer.
- Chemotherapy-Induced Thrombotic Microangiopathy in a Pediatric Patient With a Single Functioning Kidney: A Case Report and Review of the Literature.
- When Metabolism Recovers but the Brain Does Not: A Case Report of Posterior Reversible Encephalopathy Syndrome (PRES)-Like Encephalopathy After Extreme Metformin-Associated Lactic Acidosis.
- An information intervention in India reduces consumption of arsenic-contaminated drinking water and has small effects on health.
- Multi-Epitope mRNA Vaccine Targeting Dabie Bandavirus Glycoprotein: An Immunoinformatics-Based Study.
- Treatment of toxic epidermal necrolysis in a patient with extensive skin damage induced by dimethyl N-cyanodithioiminocarbonate: a case report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:293815(Orphanet)
- MONDO:0017396(MONDO)
- GARD:21171(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55787028(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
