A Necrólise Epidérmica Tóxica (NET), também conhecida como TEN, é uma doença de pele aguda e grave. Ela se caracteriza por sinais visíveis no paciente e alterações notadas em exames de tecido (histologia), onde acontece a destruição e o descolamento da camada mais superficial da pele e das mucosas (como as da boca e dos olhos).
Introdução
O que você precisa saber de cara
A Necrólise Epidérmica Tóxica (NET), também conhecida como TEN, é uma doença de pele aguda e grave. Ela se caracteriza por sinais visíveis no paciente e alterações notadas em exames de tecido (histologia), onde acontece a destruição e o descolamento da camada mais superficial da pele e das mucosas (como as da boca e dos olhos).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 36 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 94 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
3 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
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
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.
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 — Espectro clínico de síndrome de Stevens-Johnson/necrólise epidérmica tóxica
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
Ensaios em destaque
🟢 Recrutando agora
3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
41 ensaios clínicos encontrados, 6 ativos.
Publicações mais relevantes
Long-term morbidity and quality of life in survivors of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.
Feline immune-mediated skin disorders: Part 1.
Cutaneous immune-mediated diseases in cats can present with clinical signs of erythema, hair loss, scaling/crusting and erosions with or without pruritis, mimicking many differential diagnoses such as hypersensitivity dermatitis and infections (eg, parasitic and fungal dermatitis). Veterinarians are encouraged to become familiar with the characteristic clinical features of various immune-driven skin disorders to permit early diagnosis and appropriate treatment. Diagnosis and therapeutic management of feline immune-mediated skin diseases can be challenging. Although immune-mediated skin diseases in cats are rare, these can be associated with severe systemic clinical signs, which can lead to poor quality of life and owners electing euthanasia. In the first of a two-part article series, the clinical presentations of feline immune-mediated skin diseases related to the pemphigus complex, cutaneous lupus erythematosus, and thymoma- and non-thymoma-associated exfoliative dermatitis are reviewed and differential diagnoses, diagnosis and therapeutic approach are discussed. In Part 2, the equivalent is discussed for erythema multiforme, f Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum, plasma cell pododermatitis, proliferative and necrotizing otitis externa, pseudopelade, alopecia areata and auricular chondritis. The information in this article is based on a review of the existing veterinary literature.
Feline immune-mediated skin disorders: Part 2.
Practical relevanceCutaneous immune-mediated diseases in cats can present with clinical signs of erythema, hair loss, scaling/crusting, and erosions with or without pruritus, mimicking many differential diagnoses such as hypersensitivity dermatitis and infections (eg, parasitic and fungal dermatitis). Veterinarians are encouraged to become familiar with the characteristic clinical features of various immune-driven skin disorders to permit early diagnosis and appropriate treatment.Clinical challengesDiagnosis and therapeutic management of feline immune-mediated skin diseases can be challenging. Although immune-mediated skin diseases in cats are rare, these can be associated with severe systemic clinical signs, which can lead to poor quality of life and owners electing euthanasia.AimsIn the second of a two-part article series, the clinical presentations of the feline immune-mediated skin diseases erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum, plasma cell pododermatitis, proliferative and necrotizing otitis externa, pseudopelade, alopecia areata and auricular chondritis are reviewed and differential diagnoses, diagnosis and therapeutic approach are discussed. In Part 1, the equivalent is discussed for feline immune-mediated skin diseases related to the pemphigus complex, cutaneous lupus erythematosus, and thymoma- and non-thyoma-associated exfoliative dermatitis.Evidence baseThe information in this article is based on a review of the existing veterinary literature.
Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum reactions to immune checkpoint inhibitor therapy and safety of rechallenge: A retrospective review.
Drugs and the skin: A concise review of cutaneous adverse drug reactions.
Drug-induced skin disease or cutaneous adverse drug reactions (CADRs) are terms that encompass the clinical manifestations of the skin, mucosae and adnexa induced by a drug or its metabolites. The skin is the organ most frequently affected by drug reactions, which may affect up to 10% of hospitalized patients and occur in 1-3% of multimedicated patients. Most CADRs are mild or self-resolving conditions; however, 2-6.7% of could develop into potentially life-threatening conditions. CADRs represent a heterogeneous field and can be diagnostically challenging as they may potentially mimic any dermatosis. Currently, there are between 29-35 different cutaneous drug-reaction patterns reported ranging from mild dermatitis to an extensively burnt patient. The most frequently reported are maculopapular rash, urticaria/angioedema, fixed drug eruption and erythema multiforme. Less common but more severe patterns include erythroderma, drug reaction with eosinophilia and systemic symptoms, and Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum. Almost any drug can induce a CADR, but antibiotics, nonsteroidal anti-inflammatory drugs and antiepileptics are the most frequently involved. Different mechanisms are involved in the pathogenesis of CADRs, although in some cases, these remain still unknown. CADRs could be classified in different ways: (i) type A (augmented) or type B (bizarre); (ii) immediate or delayed; (iii) immune-mediated or nonimmune-mediated; (iv) nonsevere or life-threatening; and (v) by their phenotype, including exanthematous, urticarial, pustular and blistering morphology. Recognizing a specific CADR will mostly depend on the ability of the physician to perform a detailed clinical examination, the proper description of the morphology of the skin lesions and supporting laboratory and/or skin biopsy findings.
Publicações recentes
Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum reactions to immune checkpoint inhibitor therapy and safety of rechallenge: A retrospective review.
Long-term morbidity and quality of life in survivors of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.
Feline immune-mediated skin disorders: Part 1.
Feline immune-mediated skin disorders: Part 2.
Drugs and the skin: A concise review of cutaneous adverse drug reactions.
📚 EuropePMC8 artigos no totalmostrando 7
Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum reactions to immune checkpoint inhibitor therapy and safety of rechallenge: A retrospective review.
JAAD case reportsLong-term morbidity and quality of life in survivors of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.
The British journal of dermatologyFeline immune-mediated skin disorders: Part 1.
Journal of feline medicine and surgeryFeline immune-mediated skin disorders: Part 2.
Journal of feline medicine and surgeryDrugs and the skin: A concise review of cutaneous adverse drug reactions.
British journal of clinical pharmacologyUse of Etanercept in a Series of Pediatric Patients With Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis Spectrum Disease.
JAMA dermatologyThe use of cyclosporine for Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum at the University of Louisville: A case series and literature review.
Dermatology online journalAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Espectro clínico de síndrome de Stevens-Johnson/necrólise epidérmica tóxica.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Espectro clínico de síndrome de Stevens-Johnson/necrólise epidérmica tóxica
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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.
- Long-term morbidity and quality of life in survivors of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.
- Feline immune-mediated skin disorders: Part 1.
- Feline immune-mediated skin disorders: Part 2.
- Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum reactions to immune checkpoint inhibitor therapy and safety of rechallenge: A retrospective review.
- Drugs and the skin: A concise review of cutaneous adverse drug reactions.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:95455(Orphanet)
- MONDO:0019810(MONDO)
- GARD:7743(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q1878682(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
