OBSOLETO. O lúpus eritematoso cutâneo (LEC) é uma doença autoimune que denota um espectro heterogêneo de manifestações clínicas que afetam a pele e pode ser dividido em 4 categorias: LEC agudo (LECA); LEC subagudo (LECS); LEC crônico (LECC; a forma mais diversa); e LEC intermitente (LECI). O LEC pode ocorrer isoladamente ou associado ao lúpus eritematoso sistêmico (LES).
Introdução
O que você precisa saber de cara
Visão geral
O Lúpus Eritematoso Cutâneo Raro é uma doença autoimune que afeta a pele, caracterizada por um espectro heterogêneo de manifestações clínicas. Pode ser dividido em quatro categorias principais: lúpus eritematoso cutâneo agudo (LECA), subagudo (LECS), crônico (LECC) e intermitente (LECI). A condição pode ocorrer isoladamente ou estar associada ao lúpus eritematoso sistêmico (LES).[1]
A prevalência estimada é de 1 a 5 casos para cada 10.000 pessoas na população geral.[1]
A doença pode se manifestar em qualquer faixa etária, desde o nascimento até a idade adulta avançada.[1]
Sinais e sintomas
Os sinais e sintomas variam conforme o subtipo de lúpus cutâneo. As lesões podem incluir manchas avermelhadas, descamativas ou em formato de disco, sensibilidade à luz solar (fotossensibilidade) e, em alguns casos, cicatrizes ou alterações na pigmentação da pele. A gravidade e a extensão das lesões são muito variáveis entre os pacientes.[1]
Causas genéticas
Diagnóstico
O diagnóstico é baseado na avaliação clínica das lesões de pele, na história do paciente e, frequentemente, na realização de biópsia cutânea para análise histopatológica. Exames laboratoriais podem auxiliar na diferenciação entre as formas cutâneas e o lúpus sistêmico. Não há testes genéticos específicos disponíveis para confirmar o diagnóstico.[1][2]
Tratamento e manejo
O manejo do Lúpus Eritematoso Cutâneo Raro inclui medidas de proteção solar rigorosas (uso de filtros solares de amplo espectro e roupas de proteção) e tratamento tópico ou sistêmico com medicamentos. No Brasil, o acesso a alguns tratamentos pode ser parcialmente coberto pelo Sistema Único de Saúde (SUS), conforme a avaliação médica e a disponibilidade regional.[1]
Tratamentos citados na literatura
A literatura científica menciona associações entre o Lúpus Eritematoso Cutâneo Raro e os seguintes fármacos, com base em mineração de dados (número de publicações entre parênteses): dexametasona (1), acetato de dexametasona (1) e fosfato sódico de prednisolona (1). Estas são associações observadas em estudos, e não recomendações de tratamento. Consulte sempre seu médico para decisões terapêuticas.[1]
Prognóstico e qualidade de vida
O prognóstico varia conforme a extensão das lesões e a resposta ao tratamento. Muitos pacientes apresentam melhora significativa com o manejo adequado, especialmente com a proteção solar e o uso de medicamentos. A qualidade de vida pode ser impactada pelas lesões visíveis e pela fotossensibilidade, mas o acompanhamento médico regular ajuda a controlar os sintomas e a prevenir complicações.[1]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
O **lúpus eritematoso cutâneo raro** é uma condição autoimune que provoca alterações e lesões variadas na pele. O quadro pode se manifestar de maneiras distintas, como formas agudas, subagudas, crônicas ou intermitentes, em pessoas de qualquer idade. As lesões podem aparecer apenas na pele ou surgir em conjunto com o comprometimento de outros órgãos pelo lúpus eritematoso sistêmico. No Brasil, o SUS oferece suporte com fornecimento do medicamento **azatioprina** por meio do CEAF para os casos indicados.
OBSOLETO. O lúpus eritematoso cutâneo (LEC) é uma doença autoimune que denota um espectro heterogêneo de manifestações clínicas que afetam a pele e pode ser dividido em 4 categorias: LEC agudo (LECA); LEC subagudo (LECS); LEC crônico (LECC; a forma mais diversa); e LEC intermitente (LECI). O LEC pode ocorrer isoladamente ou associado ao lúpus eritematoso sistêmico (LES).
Tem tratamento?
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
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 — Lúpus eritematoso cutâneo raro
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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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
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Respirology (Carlton, Vic.)A Case of Hydroxychloroquine Toxicity as Evidenced by Visual Field Changes.
CureusAtorvastatin-induced Lupus Erythematosus Tumidus: A Case Report and Literature Review.
Acta dermatovenerologica Croatica : ADCSystemic lupus erythematosus associated with HIV infection: a retrospective case-control study.
Clinical and experimental rheumatologyIncidence and medical costs of lupus in Spanish hospitals: a retrospective database analysis.
Orphanet journal of rare diseasesNeonatal lupus erythematosus as a rare trigger of gastrointestinal involvement in neonates.
Scientific reportsDiffuse Cutaneous Mastocytosis: A Current Understanding of a Rare Disease.
International journal of molecular sciencesExploring the Perspectives of Patients Living With Lupus: Retrospective Social Listening Study.
JMIR formative researchA Case of Subcutaneous Panniculitis-Like T-cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an HIV Patient.
CureusA rare case of cutaneous lupus erythematosus presenting with periorbital erythema and edema.
LupusNovel heterozygous TREX1 mutation in a juvenile systemic lupus erythematosus patient with severe cutaneous involvement treated successfully with Jak-inhibitors: a case report.
Frontiers in immunologyRituximab to treat prolidase deficiency due to a novel pathogenic copy number variation in PEPD.
RMD openThe realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study.
Frontiers in medicineLupus panniculitis as the initial presentation of systemic lupus erythematosus triggered by COVID-19 infection: case report and literature review.
Oxford medical case reportsChildhood-Onset Systemic Lupus Erythematosus Presenting With Concomitant Gastrointestinal Manifestation and Antiphospholipid Syndrome.
CureusCornu Cutaneum: Case Reports of Patients With a Cutaneous Horn Associated With Either a Verruca Vulgaris or an Inverted Follicular Keratosis and a Review of the Etiologies of Cutaneous Horns.
CureusAnifrolumab for Moderate and Severe Muco-Cutaneous Lupus Erythematosus: A Monocentric Experience and Review of the Current Literature.
BiomedicinesA Rare Manifestation of Discoid Lupus Erythematosus Solely in the Lower Eyelid of a Young Man.
CureusThrombosis and APS: Lessons Learned from Another Black Swan Tale.
LupusRole of the NLRP1 inflammasome in skin cancer and inflammatory skin diseases.
The British journal of dermatologyBullous Systemic Lupus Erythematosus Successfully Treated With Intravenous Immunoglobulin and Mycophenolate Mofetil.
CureusHigh-risk groups of neonatal lupus erythematosus in term infants: a birth cohort study.
European journal of pediatricsRisk Factors for Pulmonary Arterial Hypertension in Patients With Systemic Lupus Erythematosus: A Systematic Review and Expert Consensus.
ACR open rheumatologyLupus Erythematosus Profundus with Multiple Overlying Cutaneous Ulcerations: A Rare Case.
Clinical, cosmetic and investigational dermatologySubacute cutaneous lupus erythematosus with a psoriasiform presentation: A diagnostic clinical conundrum.
Clinical case reportsGuideline for allergological diagnosis of drug hypersensitivity reactions: S2k Guideline of the German Society for Allergology and Clinical Immunology (DGAKI) in cooperation with the German Dermatological Society (DDG), the Association of German Allergologists (ÄDA), the German Society for Pediatric Allergology (GPA), the German Contact Dermatitis Research Group (DKG), the German Society for Pneumology (DGP), the German Society of Otorhinolaryngology, Head and Neck Surgery, the Austrian Society of Allergology and Immunology (ÖGAI), the Austrian Society of Dermatology and Venereology (ÖGDV), the German Academy of Allergology and Environmental Medicine (DAAU), and the German Documentation Center for Severe Skin Reactions (dZh).
Allergologie selectPrevalence and Characteristics of Patients With Systemic Sclerosis Fulfilling the 2019 EULAR/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus.
Arthritis care & researchEfficacy and safety of Janus kinase inhibitors in systemic and cutaneous lupus erythematosus: A systematic review and meta-analysis.
Autoimmunity reviewsAmicrobial pustulosis of the folds: A case report of a rare variant of neutrophilic dermatosis associated with systemic lupus erythematosus.
Journal of cutaneous pathologyNon-scarring alopecia of lupus erythematosus: A comprehensive review.
Annales de dermatologie et de venereologieNeonatal lupus erythematosus manifested as a complete heart block: A case report.
Clinical case reportsReticular Erythematous Mucinosis in an African Woman with HIV Infection: Case Report and Literature Review.
Case reports in dermatologyAn Uncommon Presentation of Neonatal Lupus Erythematosus: A Case Report.
Case reports in dermatologyKikuchi-Fujimoto disease: A comprehensive review.
World journal of clinical cases[Neonatal lupus erythematosus, a clinical case].
Andes pediatrica : revista Chilena de pediatriaSystemic lupus erythematosus associated with development of macrophage activation syndrome and disseminated aspergillosis.
Canadian journal of anaesthesia = Journal canadien d'anesthesieSystemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report.
Journal of medical case reportsOcular Discoid Lupus Erythematosus: More Than what Meets The Eye.
Indian dermatology online journalNeurological and endocrinological involvement in neonatal lupus erythematosus: a retrospective study at a tertiary hospital in Eastern China.
Clinical rheumatologyInsights into the clinical presentation of juvenile systemic lupus erythematosus: the PRAGMA monocentric cohort of 177 patients.
Clinical and experimental rheumatologyThe Enigma of Autoimmunity: A Case of Subacute Sclerosing Panencephalitis in a Patient Diagnosed With Systemic Lupus Erythematosus.
CureusEarly and Late Response and Glucocorticoid-Sparing Effect of Belimumab in Patients with Systemic Lupus Erythematosus with Joint and Skin Manifestations: Results from the Belimumab in Real Life Setting Study-Joint and Skin (BeRLiSS-JS).
Journal of personalized medicineSubacute cutaneous lupus erythematosus as a rare complication of disease-modifying therapy administration in multiple sclerosis: case report.
BMC neurologyInfliximab-Induced Lupus in a Patient With Psoriatic Arthritis Who Presented With Cardiac Tamponade: A Case Report.
CureusComedonic lupus, a rare variant of chronic cutaneous lupus erythematosus (CCLE): case series and literature review.
International journal of dermatologyScleroderma-Like Lupus Panniculitis: A Case Report and Literature Review.
Clinical, cosmetic and investigational dermatologyOptimal combination of arsenic trioxide and copper ions to prevent autoimmunity in a murine HOCl-induced model of systemic sclerosis.
Frontiers in immunologyLupus panniculitis: a rare bilateral and symmetrical manifestation of cutaneous lupus erythematosus in an adolescent girl.
Dermatology reportsA 30-Year-Old Woman with a History of Autoimmune Hyperthyroidism Presenting with Fever and Oral Ulcers, Diagnosed with Discoid Lupus Erythematosus.
The American journal of case reportsActinic Lichen Planus: Significance of Dermoscopic Assessment.
CureusSystemic Lupus Erythematosus Lymphadenopathy Presenting as Kikuchi-Fujimoto Disease in an Adolescent.
CureusLenalidomide for systemic lupus erythematosus with refractory cutaneous eruptions.
Rheumatology (Oxford, England)Severe immune thrombocytopenia in two patients with systemic sclerosis.
Joint bone spineSubacute cutaneous lupus erythematosus induced by Pfizer COVID-19 vaccine.
Italian journal of dermatology and venereologyAssociation of Antiphospholipid Antibodies with Clinical Manifestations in Children with Systemic Lupus Erythematosus.
Journal of clinical medicineIntestinal Submucosal Mucinosis in a Patient With Systemic Lupus Erythematosus: A Case Report.
International journal of surgical pathologyA crack in the armor: Wolf isotopic response manifesting as cutaneous lupus.
Dermatology online journalSclerodermiform Lupus Erythematosus: A Series of Five Patients.
The Journal of clinical and aesthetic dermatology[Characteristics of the course of lupus erythematosus panniculitis in a retrospective analysis of 17 patients].
Orvosi hetilapComedogenic lupus: a rare variant of chronic cutaneous lupus erythematosus - case series.
Anais brasileiros de dermatologiaMucocutaneous diseases with manifestations in the head and neck region: 24 years of experience in a Dermatology service.
Medicina oral, patologia oral y cirugia bucalA Rare Cutaneous Manifestation: Leukocytoclastic Vasculitis after Pfizer-BioNTech COVID-19 Vaccination.
Case reports in dermatological medicineUnilateral lupus mastitis.
LupusNeonatal lupus erythematosus presenting as a non-bullous histiocytoid neutrophilic dermatosis.
Journal of cutaneous pathologySARS-CoV-2 vaccine-triggered conversion from systemic lupus erythematosus (SLE) to bullous SLE and dipeptidyl peptidase 4 inhibitors-associated bullous pemphigoid.
The Journal of dermatologyA rare case of a wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome in a patient with cutaneous lupus erythematosus after COVID-19 infection.
Journal of neurologyReactive perforating collagenosis and systemic lupus erythematosus: A rare case report.
MedicineCutaneous Involvement in Catastrophic Antiphospholipid Syndrome in a Multicenter Cohort of 65 Patients.
JAMA dermatologySubcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report.
Case reports in dermatologyKeratoderma in Systemic Lupus Erythematosus: An Unusual Cutaneous Manifestation of Disease?
CureusUncommon Non-Infectious Annular Dermatoses.
Indian journal of dermatologyMulticentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature.
Journal of clinical medicineAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Lúpus eritematoso cutâneo raro.
É de uma associação que acompanha esta doença? Fale com a gente →
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Lúpus eritematoso cutâneo raro e no aconselhamento genético da família.
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
Existe um documento oficial para "Lúpus Eritematoso Sistêmico". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.
É uma doença autoimune caracterizada por manifestações clínicas que afetam a pele. Ela se divide em quatro formas principais: aguda, subaguda, crônica e intermitente.
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). Lúpus eritematoso cutâneo raro. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/lupus-eritematoso-cutaneo-raro
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Lúpus eritematoso cutâneo raro
📋 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
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Reposicionamento
- fonte: Drug Repurposing Hub
