OBSOLETO. Uma doença de reação de hipersensibilidade do tipo II que envolve a pele do corpo.
Introdução
O que você precisa saber de cara
Visão geral
A doença autoimune com envolvimento da pele é uma condição rara na qual o sistema imunológico ataca equivocadamente células e tecidos da própria pele, gerando inflamação e lesões cutâneas. Esta página foi criada para oferecer informações claras e confiáveis sobre essa condição, sempre baseadas em fontes científicas oficiais.[1]
Sinais e sintomas
Os sintomas principais envolvem a pele, podendo incluir manchas, erupções, bolhas, coceira ou descamação. Como se trata de uma doença autoimune, os sinais podem variar de pessoa para pessoa e, em alguns casos, podem estar associados a manifestações em outros órgãos. É importante consultar um médico para avaliação individualizada.[1]
Causas genéticas
Até o momento, não há genes específicos confirmados como causa direta desta doença. A condição é classificada como uma doença autoimune, e fatores genéticos podem estar envolvidos, mas ainda não foram identificados genes causais definitivos. Pesquisas continuam em andamento para entender melhor as bases genéticas.[1][2]
Diagnóstico
O diagnóstico é baseado na avaliação clínica dos sintomas de pele, histórico médico e, quando necessário, exames complementares como biópsia de pele e exames de sangue para detectar autoanticorpos. Testes genéticos não são rotineiramente indicados, pois não há genes conhecidos associados à condição. O acompanhamento com dermatologista e reumatologista é fundamental.[1][2]
Tratamento e manejo
O tratamento visa controlar a resposta autoimune e aliviar os sintomas cutâneos. As opções podem incluir medicamentos imunossupressores, corticosteroides tópicos ou sistêmicos, e terapias biológicas, sempre sob prescrição e acompanhamento médico. Não há cobertura específica pelo SUS para esta doença (classificação: Sem cobertura SUS). O manejo deve ser individualizado, considerando a gravidade e a resposta ao tratamento.[1]
Prognóstico e qualidade de vida
O prognóstico varia conforme a gravidade da doença e a resposta ao tratamento. Muitas pessoas conseguem controlar os sintomas com acompanhamento médico regular e adesão ao plano terapêutico. A qualidade de vida pode ser impactada pelas lesões de pele e pelo tratamento, mas o suporte de uma equipe multidisciplinar (dermatologista, reumatologista, psicólogo) pode ajudar a enfrentar os desafios da condição.[1]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
O termo doença autoimune com envolvimento da pele refere-se a uma categoria médica geral para enfermidades em que a imunidade afeta o tecido cutâneo. A classificação é descrita como uma reação de hipersensibilidade do tipo II direcionada à pele, mas é considerada obsoleta nos catálogos atuais. Esta designação não consta na lista oficial de PCDTs de doenças raras do Ministério da Saúde.
OBSOLETO. Uma doença de reação de hipersensibilidade do tipo II que envolve a pele do corpo.
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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
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
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 — Doença autoimune com envolvimento da pele
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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
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Dermatology (Basel, Switzerland)A Sonidegib Experience in the Treatment of Basal Cell Carcinoma with Systemic Lupus Erythematosus: A Case Report.
Clinical, cosmetic and investigational dermatologyPatient-Reported Outcomes on Quality of Life in Older Adults with Oral Pemphigus.
Healthcare (Basel, Switzerland)Assessment of autonomic dysfunction in primary Sjögren's syndrome: A combined evaluation using sympathetic skin response and COMPASS 31.
Irish journal of medical scienceCase Report: Possible association between dermatomyositis and Trastuzumab Deruxtecan therapy of triple-negative breast cancer patient.
Frontiers in immunologyNeutrophil lymphocyte ratio as a marker of skin disease activity in older children with juvenile dermatomyositis.
Pediatric rheumatology online journalAssociation of skin-advanced glycation end products, IMT complex, and bone mineral density in patients with type 1 diabetes.
Frontiers in endocrinologyDiscovery of a PDE4B and PDE4D selective bifunctional degrader for management of chronic inflammatory disorders.
Journal of translational autoimmunityClinical phenotypes, classification, and long-term outcomes of childhood-onset Sjögren's disease into adulthood: a single-centre cohort study.
The Lancet. RheumatologyModified mesenchymal stem cells as an innovative therapeutic approach in systemic lupus erythematosus: A comprehensive review.
Modern rheumatologyKaposi sarcoma in a female patient with multiple sclerosis: a case report.
Journal of medical case reportsTranscutaneous electrical nerve stimulation for upper limb neuropathic pain in Guillain-Barré syndrome: a case report.
Pain managementOverall and by subtype epidemiology of idiopathic inflammatory myopathies among African descent patients with free access to high standard healthcare.
Seminars in arthritis and rheumatismTacrolimus associated with lower infection risk compared to conventional immunosuppressants in systemic lupus erythematosus: real-world evidence.
Clinical rheumatologyLupuslike Manifestations in Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia.
JAMA dermatologyIschemic Necrosis of Bilateral Second Toes in the Absence of Macrovascular Disease or Primary Vasculitis: A Unique Case of Systemic Sclerosis Sine Scleroderma (ssSSc).
CureusNeurological Phenotypes of SOCS1 Haploinsufficiency: Insights from Functional and Histological Investigations.
Journal of clinical immunologyElderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology.
Journal of autoimmunityDistinct cytokine signature in juvenile dermatomyositis: linking myositis-specific antibodies and clinical manifestations.
CytokineRole of protein kinase C subtype-specific signalling in regulating adhesion in human keratinocytes and skin in pemphigus.
The British journal of dermatologyInitial assessment of blood-brain barrier permeability alterations and associated clinical characteristics in patients with primary Sjögren's syndrome: Insights from DCE-MRI.
Clinical rheumatologyTargeted Therapies and Pharmacologic Advances in Mucous Membrane Pemphigoid: A Comprehensive Review.
Dermatology practical & conceptualPsoriasis and Alopecia: Unveiling the Links.
Dermatology practical & conceptualThe Effectiveness and Safety of Oral N-Acetylcysteine in Combination With Narrow-Band Ultraviolet B (NB-UVB) Phototherapy Compared With NB-UVB Phototherapy Alone in the Treatment of Vitiligo: A Pilot Study.
Health science reportsRaynaud's Phenomenon and Pericardial Effusion Requiring Management with A Pericardial Window as the only Manifestations of Systemic Sclerosis without Scleroderma for over 10 Years, after which Pulmonary Hypertension Developed.
European journal of case reports in internal medicineThe Retinoid Tamibarotene Aggravates Skin Inflammation in a Model of Bullous Pemphigoid-like Epidermolysis Bullosa Acquisita.
CellsSex-Based Differences in Systemic Sclerosis Among Egyptian Patients: Insights from a Multicenter Observational Study in a Genetically Distinct North African Mediterranean Population.
Journal of clinical medicineA new, patient-friendly, needleless methotrexate administration method using the pyro-drive jet injector in rats.
Scientific reportsAyurvedic management for Epidermolysis bullosa - A case report.
Journal of Ayurveda and integrative medicineSevere Babesiosis With Multifactorial Hemolysis and Pulmonary Complications in an Asplenic Patient at a Tertiary Cancer Center.
CureusThe power of genetics in decoding Sjögren's disease: current status and future development.
Current opinion in immunology[Connective tissue diseases: development predictors, multimorbidity variants, physical treatment methods. (Literature review)].
Voprosy kurortologii, fizioterapii, i lechebnoi fizicheskoi kulturySystemic sclerosis: pathogenic mechanisms and their implications for treatment.
Seminars in immunopathologyDrug- and vaccine induced ANCA-associated vasculitis: An overview.
Autoimmunity reviewsImpact of Concordance Between Antinuclear Antibody Indirect Immunofluorescence Patterns and Myositis Antibodies in Idiopathic Inflammatory Myopathies: Study Protocol.
Acta medica portuguesaJuvenile systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome: a case report and literature review.
Orphanet journal of rare diseasesCutaneous lupus erythematosus - from pathogenesis to targeted therapy.
Nature reviews. RheumatologyA presentation of recurrent cerebral ischemic stroke in eosinophilic granulomatosis with polyangiitis: a case report.
Journal of medical case reportsMalignancy-associated Multicentric Reticulohistocytosis Mimicking Dermatomyositis-like Features, with a Significant Ultrasound Finding.
Internal medicine (Tokyo, Japan)Tumour necrosis factor-like weak inducer of apoptosis (TWEAK) is associated with inflammation and disease severity in pemphigus.
The British journal of dermatologyCharacterizing Keratinocyte-Derived Extracellular Vesicles in UVB-Irradiated Murine Skin.
JID innovations : skin science from molecules to population healthFever and rhabdomyolysis in a recent traveler: A case report of an uncommon presentation of anti-synthetase syndrome.
SAGE open medical case reportsMacular Rash as a Presenting Symptom of Acute Q Fever: A Case Report.
Case reports in infectious diseasesAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma categoria catalogada no Orphanet sob o código ORPHA:315350 que agrupa desordens mediadas por hipersensibilidade tipo II na pele. Essa definição textual é atualmente tratada como obsoleta pelos padrões internacionais de catalogação.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
3 fontes citadas no texto · 10 publicações do grafo RarasNet (PubMed) · 3 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Doença autoimune com envolvimento da pele. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/doenca-autoimune-com-envolvimento-da-pele
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Doença autoimune com envolvimento da pele
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Dado público estruturado
- fonte: Wikidata