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Doença autoimune com envolvimento da pele
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OBSOLETO. Uma doença de reação de hipersensibilidade do tipo II que envolve a pele do corpo.

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Introdução

O que você precisa saber de cara

Mantido pelo Disease Twin100% com fonte · atualizado 10/06/2026Revisão médica pendente
Informacoes curadas por IA — podem conter imprecisoes

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]

Fontes numeradas em Referências.

Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.

💬
EM LINGUAGEM SIMPLES

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.

Informacoes curadas por IA — podem conter imprecisoes
[1] Orphanet [16] Ministério da Saúde — lista oficial de…
📋
Informacoes curadas por IA — podem conter imprecisoes

OBSOLETO. Uma doença de reação de hipersensibilidade do tipo II que envolve a pele do corpo.

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SUS: Sem cobertura SUSScore: 0%
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Entender a doença

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Sinais e sintomas

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

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Anos de pesquisa1
Últimos 10 anos200publicações
Pico2026112 papers
Linha do tempo
2025Hoje · 2026
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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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

Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença autoimune com envolvimento da pele

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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

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

When atrioventricular block paves the way to a more severe diagnosis: a case report.

European heart journal. Case reports2026 Mar

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by widespread fibrosis of skin and internal organs. Cardiac involvement is common but often subclinical, and advanced conduction abnormalities such as complete atrioventricular block are rarely the initial manifestation. A 42-year-old man presented with fatigue and episodes of syncope. Electrocardiography confirmed complete AV block. Physical examination revealed diffuse skin tightening and Raynaud's phenomenon. Immunological testing detected high-titre antinuclear antibodies and anti-topoisomerase I, confirming diffuse cutaneous SSc. Further evaluation disclosed multiorgan involvement: high-resolution CT showed pulmonary fibrosis and right heart catheterization confirmed severe precapillary pulmonary hypertension. The patient underwent permanent dual-chamber pacemaker implantation and was started on immunosuppressive therapy and pulmonary vasodilators. On follow-up, his bradycardia resolved and exercise tolerance improved, indicating partial clinical improvement. This case highlights an atypical cardiac onset of diffuse SSc with high-grade conduction disease. It underscores the importance of considering systemic autoimmune disorders in patients with unexplained high-degree AV block, especially in the presence of characteristic skin findings. Comprehensive assessment revealed concurrent pulmonary and gastrointestinal involvement, reflecting the multisystem nature of SSc. Early recognition allowed a coordinated multidisciplinary treatment strategy, including device implantation and disease-modifying therapy, which ultimately stabilized the patient's condition. This case emphasizes the need for early diagnosis and integrated management of cardiac, pulmonary, and gastrointestinal complications in SSc. Although SSc is generally progressive, prompt recognition and treatment of organ involvement may significantly improve patient outcomes and quality of life.

#2

Dermatomyositis masquerading as angioedema: a crucial differential not to overlook.

BMJ case reports2026 Mar 04

Dermatomyositis is a rare autoimmune disease that typically affects the skin, muscle and respiratory system. Patients with dermatomyositis usually require prompt treatment with intense immunosuppression to reduce disease progression and multiorgan damage. A previously well male in his 40s initially presented to an emergency department with severe neck swelling. He was reviewed initially by ear, nose and throat surgeons for a probable deep neck space infection. Having been cleared of this as a cause, he was referred to clinical immunology for investigation and treatment of suspected idiopathic angioedema. A creatine kinase level was promptly requested due to the atypical nature of neck oedema that was inconsistent with angioedema, along with the presence of skin and nail changes. The patient was given a provisional diagnosis of dermatomyositis and subsequently was shown to have a positive small ubiquitin-like modifier-1 activating enzyme (SAE1) autoantibody and typical muscle biopsy. This case highlights non-histamine and non-bradykinin-mediated neck swelling as an unusual presentation of dermatomyositis that manifested with dysphagia and significant muscle involvement requiring prompt treatment.

#3

Eosinophilic Fasciitis in Pediatric Patients: A Rare but Distinct Autoimmune Fibrosing Disorder.

Pediatrics in review2026 Mar 01

Eosinophilic fasciitis (EF), or Shulman syndrome, first described in 1974, is a rare fibrosing disorder characterized by painful, symmetric swelling and progressive woody induration of skin and subcutaneous tissues. Although the pathogenesis remains unclear, EF is considered immune-mediated, often triggered by physical exertion, infections, or medications. Pediatric EF, a particularly uncommon subset, can exhibit distinct clinical features, including pronounced extracutaneous manifestations, unpredictable disease progression, and variable therapeutic responses. Clinical presentations range from rapidly advancing fibrosis leading to joint contractures to fluctuating inflammatory episodes. Diagnosis is challenging because of the absence of universal criteria, although peripheral eosinophilia, elevated inflammatory markers, and imaging findings support clinical suspicion. Definitive diagnosis depends on deep skin and fascial biopsy, revealing eosinophil-rich lymphoplasmacytic infiltrates and fibrosis. Management primarily relies on systemic corticosteroids, supplemented by steroid-sparing immunosuppressive medications in refractory cases. Early diagnosis and treatment are critical because untreated EF can cause irreversible fibrosis and significant functional impairment. The disease's rarity, heterogeneous presentations, and unclear etiology further complicate clinical management. Recent insights suggest EF may involve intricate interactions among environmental triggers, immune dysregulation, and fibrotic remodeling. This review aims to provide an updated overview of pediatric EF, highlighting current knowledge on clinical manifestations, diagnosis, differential diagnosis, therapeutic approaches, and outcomes, supported by an illustrative case, with emphasis on areas needing further research.

#4

Genome-wide association analyses of autoimmune hypothyroidism reveal autoimmune and thyroid-specific contributions and an inverse relationship with cancer risk.

Nature genetics2026 Mar

The high prevalence (>5%) of autoimmune hypothyroidism (AIHT) provides a unique opportunity to dissect genetic contributions to systemic and organ-specific autoimmunity. Here we performed a genome-wide association meta-analysis of 81,718 AIHT cases in FinnGen and the UK Biobank, identifying 418 independent signals (P < 5 × 10-8). At 48 of these loci, a protein-coding variant is, or is highly correlated (r2 > 0.95) with, the lead variant, including Finnish-enriched coding variants in LAG3, ZAP70 and TG. We demonstrated that ZAP70:T155M reduces T cell activation and broadly compare large-scale scans of nonthyroid autoimmunity and thyroid-stimulating hormone levels with a Bayesian classifier to assign loci into distinct groupings, estimating that 38% are involved in general autoimmunity whereas 20% are thyroid specific. We further identified substantial antagonistic pleiotropy, with 10% of AIHT loci showing a consistent protective effect against skin cancer. The AIHT results, including numerous genes encoding checkpoint proteins, support the causal role of natural immune variation influencing cancer outcomes.

#5

Acute rheumatic fever and rheumatic heart disease.

Nature reviews. Disease primers2026 Feb 26

Acute rheumatic fever (ARF), the acute autoimmune sequela of group A Streptococcus infection, and its chronic life-threatening manifestation, rheumatic heart disease (RHD), which causes permanent heart valve damage, remain major drivers of preventable cardiovascular mortality and disability in low-income and middle-income countries. Since the mid-1990s, multidisciplinary and international collaborative research has yielded new insights into the role of superficial skin infections such as impetigo in ARF pathogenesis and the disease mechanisms that underlie the progression from ARF to RHD, and has explored novel treatments and delivery mechanisms in clinical trials and progressed the development of Strep A vaccines. Early detection and patient care have advanced, with revised echocardiographic screening criteria and the 2024 WHO guidelines, which provide a framework for evidence-based management and therapeutic strategies. Notwithstanding these advancements, considerable disparity persists in the care, control and treatment of advanced disease, necessitating coordinated programmes, prioritization by global agencies and local governments, and substantial involvement of those with RHD. Of note, few to no cardiac surgery programmes exist in some countries most burdened by RHD. Future research must encompass implementation studies for validated evidence-based therapies, translational technologies and applications, together with a definitive pathway to direct patient effect, to achieve the WHO target of eliminating RHD as a public health problem.

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Persistent cutaneous lupus erythematosus: A pathway toward systemic disease?

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When atrioventricular block paves the way to a more severe diagnosis: a case report.

European heart journal. Case reports
2026

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Frontiers in medicine
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Quality control strategy for differentiating induced pluripotent stem cells into pancreatic β-cells before transplantation.

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Adult-Onset Still's Disease Presenting with Progressive Motor Neuropathy.

European journal of case reports in internal medicine
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Comprehensive Analysis Reveals Potential Molecular Targets in Juvenile Dermatomyositis.

Biochemistry research international
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Incidence of P200 pemphigoid: A nationwide study.

Journal of the European Academy of Dermatology and Venereology : JEADV
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Genomic and proteomic insights into hidradenitis suppurativa.

Journal of the European Academy of Dermatology and Venereology : JEADV
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Longitudinal Assessment of the Juvenile Systemic Sclerosis Severity Score: Application in the National Registry for Childhood Onset Scleroderma.

ACR open rheumatology
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A retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.

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Cutaneous Rosai-Dorfman disease: a case report of disease limited only to skin.

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Exploring the anti-fibrotic effects of safflower in systemic sclerosis based on metabolomics and gut microbiota analyses.

Frontiers in pharmacology
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The clinical phenotype of anti-Th/To+ patients in systemic sclerosis: a case-control study within the European Scleroderma Trials and Research cohort.

Therapeutic advances in musculoskeletal disease
2026

Dermatomyositis masquerading as angioedema: a crucial differential not to overlook.

BMJ case reports
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Soluble Fas Ligand, an overlooked target of therapy in dermatological and non-dermatological conditions.

The Journal of dermatological treatment
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Lymphocytic Vasculopathy With Cytoplasmic Antineutrophil Cytoplasmic Antibody Positivity After Upper Respiratory Infection: A Case Report.

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Redefining evidence for teprotumumab in thyroid eye disease: an updated meta-analysis of efficacy and safety.

Frontiers in endocrinology
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Eosinophilic Fasciitis in Pediatric Patients: A Rare but Distinct Autoimmune Fibrosing Disorder.

Pediatrics in review
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Clinical, serological, and targeted genetic analysis of systemic lupus erythematosus in Kazakhstan.

Lupus
2026

Genome-wide association analyses of autoimmune hypothyroidism reveal autoimmune and thyroid-specific contributions and an inverse relationship with cancer risk.

Nature genetics
2026

Acute rheumatic fever and rheumatic heart disease.

Nature reviews. Disease primers
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Tackling the diagnosis of HA20 in children: challenges of a highly variable clinical and genetic spectrum.

RMD open
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Idiopathic inflammatory myopathies: one year in review 2025.

Clinical and experimental rheumatology
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Sex-based clinical and immunological differences across lupus erythematosus subtypes: a cross-sectional multicentre study from China.

Lupus science &amp; medicine
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An atypical case of granulomatosis with polyangiitis in a 15-year-old girl presenting with pyoderma gangrenosum-like ulcers on the face.

Dermatology online journal
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Pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis.

Seminars in arthritis and rheumatism
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Evaluation of Helicobacter pylori infection and clinical features in multiple sclerosis patients coexisting with vitiligo: Case-series.

Journal of the National Medical Association
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Caspase-activation powers anti-Desmoglein 3-induced acantholysis in human epidermis.

Cell death discovery
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Cytometry-based blood immune cell markers associated with clinical outcomes in systemic sclerosis: protocol for a systematic review.

BMJ open
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Development and optimization of sulfasalazine loaded microemulsion for improved topical treatment of psoriasis.

Therapeutic delivery
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An Under-Recognized Manifestation of Systemic Sclerosis: Macrovascular Peripheral Arterial Disease.

Cureus
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Anti-MDA5 Antibody-Positive Dermatomyositis in Emirati Patients: A Case Series Highlighting Clinical Variability.

The American journal of case reports
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Case Report: Extracorporeal photopheresis for cutaneous lupus erythematosus induces putatively atheroprotective B and T cell responses.

Frontiers in immunology
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Successful use of upadacitinib for the improvement of vitiligo-like and perifollicular dyspigmentation (salt-and-pepper skin) in systemic sclerosis.

Dermatology reports
2026

Pathogenic and Clinical Relevance of Serum IL-17A and TNF-α in Systemic Lupus Erythematosus.

International journal of molecular sciences
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Anifrolumab-A Potential New Systemic Sclerosis Treatment.

Journal of clinical medicine
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Bullous Pemphigoid Following Total Knee Arthroplasty: A Case Report.

JBJS case connector
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Assessment of myositis specific antibodies in primary chronic arthritis: single centre prospective study.

Clinical and experimental rheumatology
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A case report of IgA-mediated anti-Laminin-γ1 (p200) pemphigoid successfully treated with stapokibart combined with corticosteroids.

Frontiers in immunology
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Vogt-Koyanagi-Harada Disease in Paediatric Population: Case Series and Literature Review.

Ocular immunology and inflammation
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Early detection and intervention in dermatomyositis: current evidence and clinical implications.

Clinical rheumatology
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Analysis of the Dynamics of Clinical Parameters and Serum Angiogenic Factors in Systemic Sclerosis Patients Undergoing Tocilizumab Treatment.

Experimental dermatology
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Beneath the Cure Lies the Risk: Carbimazole-Induced Angioedema in Graves' Disease.

Cureus
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Age and onset timing of Raynaud's phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR).

Therapeutic advances in musculoskeletal disease
2026

[Fasciitis-like primary breast pyoderma gangrenosum: A rare case report].

Annales de chirurgie plastique et esthetique
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Prevalence of sleep disorders in patients with bullous pemphigoid.

Annales de dermatologie et de venereologie
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Mechanistic insights into sitagliptin-induced skin autoimmunity: Linking environmental drug exposure to IL-6 activation in bullous pemphigoid.

Ecotoxicology and environmental safety
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Durability of Conventional Immunosuppressants in the Treatment of Oral Lichen Planus.

The Journal of clinical and aesthetic dermatology
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The Gut Microbiota: Emerging Evidence in Autoimmune and Inflammatory Diseases.

Research (Washington, D.C.)
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Hypoxia and oxidative stress related to inflammation and vascular aspects of the pathogenesis of psoriasis.

Skin health and disease
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Identifying distinct clinical phenotypes and outcomes in adult-onset IgA vasculitis using unsupervised clustering analysis.

European journal of internal medicine
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Juvenile systemic sclerosis.

Best practice &amp; research. Clinical rheumatology
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Efficacy and safety of autologous CD5-KO anti-CD5 CAR-T cells in relapsed/refractory CD5+ hematological malignancies.

Cell reports. Medicine
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Myocardial involvement in inflammatory myopathies: from subclinical disease to life-threatening myocarditis.

Expert review of clinical immunology
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Dimethyl fumarate as a versatile therapeutic agent: molecular mechanisms and potential clinical applications.

Molecular biology reports
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Omalizumab Therapy for Bullous Pemphigoid: A Case Report.

Cureus
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Long-pulsed Erbium: YAG laser for the treatment of an adult colloid milium: a case report and review of the literature.

Journal of cosmetic and laser therapy : official publication of the European Society for Laser Dermatology
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Vaccine-associated lupus and related organ involvements: A pharmacovigilance analysis of VAERS database 1990-2025.

Seminars in arthritis and rheumatism
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Fc-enhanced anti-CCR6 antibody elicits robust therapeutic effects across multiple autoimmune diseases.

Frontiers in immunology
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Psoriatic Arthritis: Developments in 2025.

Mediterranean journal of rheumatology
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Complex Dermatological Manifestations of Poorly Controlled Diabetes: A Case of Acquired Ichthyosis.

Case reports in dermatological medicine
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Predictors of early treatment response to antihistamines and omalizumab in chronic spontaneous urticaria.

Frontiers in allergy
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JAK inhibitors for the treatment of juvenile localized scleroderma: a case report and literature review.

Pediatric rheumatology online journal
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Hypocomplementemic Urticarial Vasculitis: A Case Report.

Cureus
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Pediatric Psoriasis: A Peek into the Comorbidity Profile.

Indian journal of dermatology
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Prevalence and severity of complicated Raynaud's phenomenon in limited and diffuse systemic sclerosis: a multicenter study in Iraq.

Reumatologia
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Efgartigimod as a treatment for people with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a plain language summary of publication of the ADHERE trial.

Therapeutic advances in neurological disorders
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Low Vitamin D and C Levels are Associated with Increased Disease Activity in Jordanian RA and SLE Patients: A Case-Control Study.

Endocrine, metabolic &amp; immune disorders drug targets
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[From a simple skin rash to kidney injury: A case of post-measles glomerulonephritis].

Nephrologie &amp; therapeutique
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Litifilimab efficacy on skin outcomes in cutaneous lupus erythematosus in the Phase 2 LILAC study.

Journal of the European Academy of Dermatology and Venereology : JEADV
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A Pathway to High Quality Clinical Trials in IgA Vasculitis Nephritis: Meeting Proceedings From a Multiprofessional International Collaborative Workshop.

Kidney international reports
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Case Report: Refractory hidradenitis suppurativa complicated by IgA vasculitis with nephritis: successful long-term control with surgery and bimekizumab.

Frontiers in immunology
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Case report: Epidermolysis bullosa acquisita following dipeptidyl peptidase-4 inhibitor therapy and complicated by immune thrombocytopenic purpura.

Frontiers in immunology
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New-onset refractory status epilepticus associated with Sjögren's disease.

BMJ neurology open
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Allgrove syndrome with early neurodegeneration in a child: A case report from Syria.

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Immunoglobulin G4-related disease with multi-organ involvement: A case report.

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Impact of scalp involvement on disease severity and professional life in patients with cutaneous lupus erythematosus.

Lupus
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Bullous Diseases in the Perioperative Setting: Anesthetic Considerations.

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NGO ameliorates psoriasis by modulating mitochondrial function and suppressing pSTAT3-IL-17-expressing CD8+ TRM cells.

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Lower Extremity Salvage With Free Tissue Transfer in the Setting of Bullous Pemphigoid.

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Paraneoplastic Pemphigus in a Patient With Relapsed Breast Cancer: An Uncommon Presentation.

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Comparative Efficacy of Autologous Hematopoietic and Mesenchymal Stem Cell Transplantation in Patients with Systemic Sclerosis: A Systematic Review.

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Risk of major cardiovascular events in patients with systemic sclerosis: insights into an underestimated concern from a systematic literature review and meta-analysis.

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Antimicrobial resistance of Group A Streptococcus isolates from patients in Shenzhen, China during COVID-19 pandemic.

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Development and in vitro/in vivo evaluation of polymeric dissolving microneedle formulation for psoriasis treatment.

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Nailfold videocapillaroscopy in the assessment of juvenile connective tissue diseases.

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Lyme disease as a rare trigger for autoimmune hemolytic anemia.

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Frontiers in immunology
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Anti-Inflammatory Effect of Urtica urens on LPS-Challenged Keratinocytes.

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Subacute cutaneous lupus erythematosus in a patient with Whipple's disease.

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Pyoderma gangrenosum associated with granulomatosis with polyangiitis: a case report.

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BDJ open
2025

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Endocrinologia, diabetes y nutricion
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The British journal of dermatology
2025

Behçet's Disease Mimicking Drug-Induced Mucocutaneous Lesions in a Patient on Anti-tuberculous Drugs.

Cureus
2026

Review of literature: posterior reversible encephalopathy syndrome in systemic lupus erythematosus.

Journal of rheumatic diseases
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
2025

Macrophage activation syndrome in a child with varicella: a case report.

Journal of medical case reports
2025

[Impact of aspirin use on pregnancy outcomes in patients with systemic lupus erythematosus].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2026

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

Inflammopharmacology
2025

What's the connection? Sarcoidosis and combined variable immunodeficiency: A Single center retrospective case series.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
2026

Insights from a novel monogenic autoinflammatory disease: overview of a multicentric European cohort of 38 patients with COPA syndrome.

Annals of the rheumatic diseases
2025

Case Report: VEXAS syndrome with extensive pulmonary, cardiac, and skeletal involvement.

Frontiers in immunology
2026

Prevalence, distribution and associations of the scleroderma capillaroscopic patterns: new insights from the Italian SPRING-SIR registry.

Rheumatology (Oxford, England)
2025

Ophthalmologic Comorbidities in Alopecia Areata.

Journal of clinical medicine
2026

Ruxolitinib in the Treatment of Vitiligo. The Importance of the JAK STAT Pathway.

Actas dermo-sifiliograficas
2026

[Preexisting diabetes: Expert consensus from the College of French Gynecologists and Obstetricians and from the French Society of Diabetology].

Gynecologie, obstetrique, fertilite &amp; senologie
2025

Band Acro-Osteolysis as a Less Common Radiologic Pattern in Systemic Scleroderma: A Case Report.

Clinical medicine insights. Case reports
2025

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

Cureus
2025

A case report of eosinophilic granulomatosis with polyangiitis in children with cerebella infarction as the first symptom and literature review.

Frontiers in immunology
2025

Endothelial cell-targeting aptamer-empowered exosomes accelerate wound healing by promoting specialized angiogenesis in type 1 diabetic mice.

Stem cell research &amp; therapy
2025

Salivary Oxidative Stress Markers in Behcet's: A Comparative Study of Active and Inactive Diseases.

Health science reports
2025

Gastric Antral Vascular Ectasia Preceding the Diagnosis of Limited Cutaneous Systemic Sclerosis.

Cureus
2025

Biopharmaceuticals: importance, application, function - a narrative review.

European review for medical and pharmacological sciences
2025

Metastatic Malignancy Masquerading as Skin Rash.

Cureus
2026

SpeckSeq enables high-throughput functional stratification of MEFV variants in autoinflammatory diseases.

The Journal of experimental medicine
2025

An Immunocompetent Host With Severe Disseminated Herpes Simplex Virus Type 2 Infection With Hepatorenal Involvement.

Cureus
2025

Dermatomyositis and microscopic polyangiitis overlap: a case-based review.

Rheumatology international
2026

Single-cell RNA sequencing and proteomics uncover glycolytic dysregulation linking skin and systemic inflammation in dermatomyositis.

The British journal of dermatology
2025

A Rare Case of Bullous Pemphigoid Triggered by Overlapping Immune Dysregulation From HIV and Hodgkin Lymphoma: A Case Report.

Cureus
2026

Pathogenesis of Vitiligo: Integrating Immune and Non-Immune Cell Crosstalk.

The Journal of dermatology
2025

Parry-Romberg Syndrome With Localized Scleroderma: A Report of Two Pediatric Cases From Oman.

Cureus
2025

Overlap of Toxic Epidermal Necrolysis (TEN)-Like Cutaneous Lupus and Rowell Syndrome in Systemic Lupus Erythematosus: A Case-Based Review.

Cureus
2025

The hidden burden of visibility: Social appearance anxiety in patients with alopecia areata - A comparative cross-sectional study.

Journal of health psychology
2025

Mixed Cryoglobulinemia in the Setting of Monoclonal B-Cell Lymphocytosis and Monoclonal Gammopathy of Unknown Significance: A Case Report.

The American journal of case reports
2025

Systemic sclerosis: bridging clinical and molecular insights: results from the PRECISESADS study.

Journal of translational medicine
2026

Pathogenesis of skin damage in lupus: recent advances and future directions.

Current opinion in rheumatology
2025

Autologous Hematopoietic Stem Cell Transplantation in Systemic Sclerosis: Current Evidence and Future Directions.

ACR open rheumatology
2026

The Patient Journey in Vitiligo.

Dermatology (Basel, Switzerland)
2025

A Sonidegib Experience in the Treatment of Basal Cell Carcinoma with Systemic Lupus Erythematosus: A Case Report.

Clinical, cosmetic and investigational dermatology
2025

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

Healthcare (Basel, Switzerland)
2026

Assessment of autonomic dysfunction in primary Sjögren's syndrome: A combined evaluation using sympathetic skin response and COMPASS 31.

Irish journal of medical science
2025

Case Report: Possible association between dermatomyositis and Trastuzumab Deruxtecan therapy of triple-negative breast cancer patient.

Frontiers in immunology
2025

Neutrophil lymphocyte ratio as a marker of skin disease activity in older children with juvenile dermatomyositis.

Pediatric rheumatology online journal
2025

Association of skin-advanced glycation end products, IMT complex, and bone mineral density in patients with type 1 diabetes.

Frontiers in endocrinology
2025

Discovery of a PDE4B and PDE4D selective bifunctional degrader for management of chronic inflammatory disorders.

Journal of translational autoimmunity
2026

Clinical phenotypes, classification, and long-term outcomes of childhood-onset Sjögren's disease into adulthood: a single-centre cohort study.

The Lancet. Rheumatology
2026

Modified mesenchymal stem cells as an innovative therapeutic approach in systemic lupus erythematosus: A comprehensive review.

Modern rheumatology
2025

Kaposi sarcoma in a female patient with multiple sclerosis: a case report.

Journal of medical case reports
2026

Transcutaneous electrical nerve stimulation for upper limb neuropathic pain in Guillain-Barré syndrome: a case report.

Pain management
2025

Overall and by subtype epidemiology of idiopathic inflammatory myopathies among African descent patients with free access to high standard healthcare.

Seminars in arthritis and rheumatism
2026

Tacrolimus associated with lower infection risk compared to conventional immunosuppressants in systemic lupus erythematosus: real-world evidence.

Clinical rheumatology
2026

Lupuslike Manifestations in Myelodysplastic Syndromes and Chronic Myelomonocytic Leukemia.

JAMA dermatology
2025

Ischemic Necrosis of Bilateral Second Toes in the Absence of Macrovascular Disease or Primary Vasculitis: A Unique Case of Systemic Sclerosis Sine Scleroderma (ssSSc).

Cureus
2025

Neurological Phenotypes of SOCS1 Haploinsufficiency: Insights from Functional and Histological Investigations.

Journal of clinical immunology
2025

Elderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology.

Journal of autoimmunity
2026

Distinct cytokine signature in juvenile dermatomyositis: linking myositis-specific antibodies and clinical manifestations.

Cytokine
2026

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

The British journal of dermatology
2026

Initial assessment of blood-brain barrier permeability alterations and associated clinical characteristics in patients with primary Sjögren's syndrome: Insights from DCE-MRI.

Clinical rheumatology
2025

Targeted Therapies and Pharmacologic Advances in Mucous Membrane Pemphigoid: A Comprehensive Review.

Dermatology practical &amp; conceptual
2025

Psoriasis and Alopecia: Unveiling the Links.

Dermatology practical &amp; conceptual
2025

The 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 reports
2025

Raynaud'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 medicine
2025

The Retinoid Tamibarotene Aggravates Skin Inflammation in a Model of Bullous Pemphigoid-like Epidermolysis Bullosa Acquisita.

Cells
2025

Sex-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 medicine
2025

A new, patient-friendly, needleless methotrexate administration method using the pyro-drive jet injector in rats.

Scientific reports
2025

Ayurvedic management for Epidermolysis bullosa - A case report.

Journal of Ayurveda and integrative medicine
2025

Severe Babesiosis With Multifactorial Hemolysis and Pulmonary Complications in an Asplenic Patient at a Tertiary Cancer Center.

Cureus
2026

The power of genetics in decoding Sjögren's disease: current status and future development.

Current opinion in immunology
2025

[Connective tissue diseases: development predictors, multimorbidity variants, physical treatment methods. (Literature review)].

Voprosy kurortologii, fizioterapii, i lechebnoi fizicheskoi kultury
2025

Systemic sclerosis: pathogenic mechanisms and their implications for treatment.

Seminars in immunopathology
2026

Drug- and vaccine induced ANCA-associated vasculitis: An overview.

Autoimmunity reviews
2025

Impact of Concordance Between Antinuclear Antibody Indirect Immunofluorescence Patterns and Myositis Antibodies in Idiopathic Inflammatory Myopathies: Study Protocol.

Acta medica portuguesa
2025

Juvenile systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome: a case report and literature review.

Orphanet journal of rare diseases
2025

Cutaneous lupus erythematosus - from pathogenesis to targeted therapy.

Nature reviews. Rheumatology
2025

A presentation of recurrent cerebral ischemic stroke in eosinophilic granulomatosis with polyangiitis: a case report.

Journal of medical case reports
2025

Malignancy-associated Multicentric Reticulohistocytosis Mimicking Dermatomyositis-like Features, with a Significant Ultrasound Finding.

Internal medicine (Tokyo, Japan)
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

Characterizing Keratinocyte-Derived Extracellular Vesicles in UVB-Irradiated Murine Skin.

JID innovations : skin science from molecules to population health
2025

Fever and rhabdomyolysis in a recent traveler: A case report of an uncommon presentation of anti-synthetase syndrome.

SAGE open medical case reports
2025

Macular Rash as a Presenting Symptom of Acute Q Fever: A Case Report.

Case reports in infectious diseases

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Conselhos e sociedades

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

Respostas geradas por IA a partir das fontes citadas

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.

  1. MONDO
    purl.obolibrary.org
  2. Q12504769
    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.

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

Compêndio · Raras BR

Doença autoimune com envolvimento da pele

ORPHA:315350 · MONDO:0017841
MedGen
UMLS
C0406632
Wikidata

📋 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
Rara