Um tipo de lúpus eritematoso caracterizado por nódulos dérmicos profundos ou subcutâneos, mais frequentemente na cabeça, face ou braços. Geralmente é crônico e ocorre com mais frequência em mulheres entre 20 e 45 anos de idade.
Introdução
O que você precisa saber de cara
Visão geral
O lúpus eritematoso paniculite (também identificado pelo código MONDO:0019561 e CID-10 L93.2) é um tipo de lúpus eritematoso caracterizado por nódulos dérmicos profundos ou subcutâneos, que surgem mais frequentemente na cabeça, face ou braços. A condição geralmente é crônica e ocorre com maior frequência em mulheres entre 20 e 45 anos de idade.[1]
Trata-se de uma condição rara, com prevalência estimada entre 1 e 9 casos a cada 1.000.000 de pessoas. O início das manifestações clínicas pode ocorrer em diversas fases da vida, abrangendo bebês (infância precoce), crianças, adolescentes, adultos e idosos.[1]
Sinais e sintomas
Clinicamente, a doença se manifesta por meio de nódulos profundos na derme ou no tecido subcutâneo, localizados preferencialmente nas regiões da cabeça, da face ou dos membros superiores (braços).[1]
Tratamentos citados na literatura
A literatura biomédica indexada no PubTator3 reúne menções científicas associadas à doença, não consistindo em diretrizes ou recomendações terapêuticas. Os compostos citados e seus respectivos números de publicações são: hidroxicloroquina (Hydroxychloroquine, 20 publicações), prednisolona (Prednisolone, 9 publicações), cloroquina (Chloroquine, 8 publicações), dapsona (Dapsone, 7 publicações), rituximabe (Rituximab, 7 publicações), ciclosporina (Cyclosporine, 6 publicações), metotrexato (Methotrexate, 6 publicações), esteroides em geral (Steroids, 6 publicações), prednisona (Prednisone, 5 publicações) e talidomida (Thalidomide, 5 publicações). Foram mapeados também estudos de reposicionamento envolvendo dexametasona, acetato de dexametasona e fosfato sódico de prednisolona.[2]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
O **lúpus eritematoso paniculite** é uma condição rara e crônica que atinge a camada de gordura sob a pele. Ele costuma provocar o aparecimento de nódulos ou caroços profundos, principalmente na face, na cabeça ou nos braços. Embora possa se iniciar em qualquer idade, afeta mais comumente mulheres entre os 20 e 45 anos. No Sistema Único de Saúde (SUS), o medicamento azatioprina é dispensado por meio do CEAF para auxílio no manejo terapêutico.
Um tipo de lúpus eritematoso caracterizado por nódulos dérmicos profundos ou subcutâneos, mais frequentemente na cabeça, face ou braços. Geralmente é crônico e ocorre com mais frequência em mulheres entre 20 e 45 anos de idade.
Tem tratamento?
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
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 paniculite
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.
Publicações mais relevantes
A Diagnostic Pitfall: Lupus Mastitis Presenting Before Diagnosis of Systemic Lupus Erythematosus.
Lupus erythematosus panniculitis or lupus erythematosus profundus (LEP) is a rare manifestation of cutaneous lupus erythematosus, with an estimated prevalence of 2%-3% in those with either systemic lupus erythematosus (SLE) or discoid lupus erythematosus (DLE). LEP with involvement of the breasts is termed lupus mastitis (LM). Its presentation is heterogenous, with epidermal changes, erythema, violaceous skin changes, lipoatrophy, and ulceration with or without breast masses. LM may resemble breast malignancy; however, the clinical course, laboratory investigations, and imaging may often differentiate these. Should uncertainty still exist, LM may be confirmed on histopathology. LM is a chronic disease, and its natural course may include exacerbations and remissions. Antimalarial agents are the mainstay of treatment, whilst corticosteroids and cyclophosphamide have demonstrated utility. There is no standardized treatment protocol. We here present the case of a 50-year-old woman who was diagnosed with SLE and LM after several indeterminate breast biopsies with the intention of furthering awareness of this presentation.
Lupus Erythematosus Panniculitis Patients With Overlying Discoid Lupus Erythematosus Have Higher Rates of Head and Neck Involvement Than Those Without.
Lupus Erythematosus Panniculitis.
The Potential of Telitacicept in Treating Lupus erythematosus panniculitis: A Case Report.
Lupus erythematosus panniculitis (LEP) is a rare and serious skin lesion associated with systemic lupus erythematosus (SLE). In the past, the management of LEP has followed the treatment guidelines for SLE, which is based on the administration of high doses of hormones together with immunosuppressants, with no treatment plans specific for LEP. However, the long-term use of high hormone doses and immunosuppressants can exacerbate skin breakage and calcification. Telitacicep is an approved biological agent demonstrating significant therapeutic efficacy in immune system disorders. We report a case of lupus erythematosus panniculitis in which the patient's clinical symptoms and laboratory test results improved after treatment with a combination of steroids, cyclophosphamide, and the biologic agent Telitacicept. The condition was significantly controlled and alleviated. This report highlights the successful application of Telitacicept in the treatment of lupus panniculitis, providing valuable insights for future management of this condition.
Subcutaneous Panniculitis-Like T-Cell Lymphoma With Increased γδ T Cells: A Potential Diagnostic Pitfall.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous T-cell lymphoma with adipotropism characterized by infiltration of CD8 + T cells exhibiting a cytotoxic and αβ phenotype. Although SPTCL is typically indolent, distinguishing it from more aggressive cutaneous lymphomas such as primary cutaneous γδ T-cell lymphoma is critical for accurate prognosis. We present a case of SPTCL in a 26-year-old Asian woman with a history of hemophagocytic lymphohistiocytosis, who presented with nonulcerated, erythematous patches and hyperpigmented nodules on her extremities and trunk persisting for 5 months. Biopsy specimens revealed an atypical lymphoid infiltrate with prominent adipotropism, predominantly comprising atypical medium-sized lymphocytes rimming the adipocytes. Immunophenotypically, the lesional lymphocytes were CD3+/CD4-/CD8+/CD7+ T cells expressing TIA1, granzyme B, and TCR βF1, while lacking CD30 and Epstein-Barr virus-encoded small RNA expression. Intriguingly, there was a markedly increased density of T cells expressing TCR δ, some surrounding adipocytes and appearing neoplastic. CD123 failed to reveal aggregates of plasmacytoid dendritic cells. Molecular analysis revealed monoclonal TCR β and γ gene rearrangements. Despite the elevated γδ T-cell population (20%-50% of the T cells), the overall clinical, histopathologic, and molecular findings, along with the absence of rapid disease progression, supported a diagnosis of SPTCL. Although increased densities of reactive γδ T-cells or γδ T-cell phenotypes in neoplastic cells are observed in various benign and malignant dermatologic conditions, the literature on this phenomenon-particularly in SPTCL-remains scarce. Our case highlights the importance of recognizing an increased γδ T-cell population in SPTCL to prevent misdiagnosis as a more aggressive lymphoma such as primary cutaneous γδ T-cell lymphoma, underscoring the need for thorough clinicopathologic correlation.
Publicações recentes
Discoid Lupus Erythematosus.
A Diagnostic Pitfall: Lupus Mastitis Presenting Before Diagnosis of Systemic Lupus Erythematosus.
Lupus Erythematosus Panniculitis Patients With Overlying Discoid Lupus Erythematosus Have Higher Rates of Head and Neck Involvement Than Those Without.
Lupus Erythematosus Panniculitis.
Childhood lupus erythematosus panniculitis.
📚 EuropePMC80 artigos no totalmostrando 68
A Diagnostic Pitfall: Lupus Mastitis Presenting Before Diagnosis of Systemic Lupus Erythematosus.
Case reports in rheumatologyLupus Erythematosus Panniculitis Patients With Overlying Discoid Lupus Erythematosus Have Higher Rates of Head and Neck Involvement Than Those Without.
The Journal of dermatologyLupus Erythematosus Panniculitis.
Journal of cutaneous medicine and surgeryChildhood lupus erythematosus panniculitis.
European journal of dermatology : EJDThe Potential of Telitacicept in Treating Lupus erythematosus panniculitis: A Case Report.
Biologics : targets & therapySubcutaneous Panniculitis-Like T-Cell Lymphoma With Increased γδ T Cells: A Potential Diagnostic Pitfall.
The American Journal of dermatopathologyAssociation of truncal involvement in lupus erythematosus panniculitis patients with calcinosis cutis: A retrospective cohort study.
LupusMesenteric Panniculitis in a Renal Transplant Patient With Systemic Lupus Erythematosus.
CureusThree-dimensional stereophotogrammetry: an effective tool for detecting disease progression in lupus erythematosus panniculitis.
Clinical and experimental dermatologyA Case of Progressive Lipoatrophy due to Lupus Erythematosus Panniculitis Induced by Quadrivalent Human Papillomavirus Vaccine.
Indian journal of dermatologyDifferent Mutational Profiles of Subcutaneous Panniculitis-like T-cell Lymphoma and Lupus Panniculitis: An Additional Case Series.
Actas dermo-sifiliograficasA double-blind pilot study of oral baricitinib in adult patients with lupus erythematosus panniculitis.
The Journal of dermatologyPrimary Cutaneous Gamma/Delta T-cell Lymphoma Simulating Lupus Erythematosus Panniculitis: What do we know about this aggressive mimicker?
Sultan Qaboos University medical journalA Case of Subcutaneous Panniculitis-Like T-cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an HIV Patient.
CureusLupus panniculitis as the initial presentation of systemic lupus erythematosus triggered by COVID-19 infection: case report and literature review.
Oxford medical case reportsLupus Erythematosus Panniculitis: A Rare Case Report.
International medical case reports journalAnifrolumab for refractory lupus erythematosus panniculitis in systemic lupus erythematosus.
Rheumatology (Oxford, England)IRF8 in Conjunction With CD123 and CD20 to Distinguish Lupus Erythematosus Panniculitis From Subcutaneous Panniculitis-like T-Cell Lymphoma.
The American journal of surgical pathologyLupus mastitis and antiphospholipid syndrome treated with anticoagulation and immunosuppression: a case report.
Journal of medical case reportsScleroderma-Like Lupus Panniculitis: A Case Report and Literature Review.
Clinical, cosmetic and investigational dermatologyExacerbation of Lupus Erythematosus Panniculitis After Administration of COVID-19 mRNA Vaccine.
Indian journal of dermatology[Characteristics of the course of lupus erythematosus panniculitis in a retrospective analysis of 17 patients].
Orvosi hetilapSubcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report.
Case reports in dermatologyCytophagic Histiocytic Panniculitis and Kikuchi-Fujimoto-Like Lymphadenitis in a Patient With Systemic Lupus Erythematosus.
CureusCentrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: A clinicopathological study.
Dermatologic therapyChronic Cutaneous Lupus Erythematosus in a White Population: Dermoscopic Characteristics by Clinical Subtype, Lesion Location and Disease Duration.
Dermatology and therapyPrimary cutaneous gamma-delta T-cell lymphoma masquerading as leukemia cutis in a patient recently diagnosed with small lymphocytic lymphoma: Clues to the diagnosis.
Journal of cutaneous pathologyOrbital Mass as the Only Presenting Sign with Overlapping Features of Lupus Erythematosus Panniculitis and Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Ocular immunology and inflammationA rare case of suspected lupus erythematous panniculitis as the presenting skin feature of juvenile dermatomyositis: A case report.
SAGE open medical case reportsPediatric-Onset Refractory Lupus Erythematosus Panniculitis Treated With Rituximab.
CutisDiffuse painful erythema on right thigh.
JAAD case reportsSubcutaneous panniculitis-like T cell lymphoma arising in association with chronic lymphocytic leukaemia.
BMJ case reportsNovel use of poly-L-lactic acid filler for the treatment of facial cutaneous atrophy in patients with connective tissue disease.
Journal of cosmetic dermatologySubcutaneous panniculitis-like T-cell lymphoma, lupus erythematosus profundus, and overlapping cases: molecular characterization through the study of 208 genes.
Leukemia & lymphomaUltrasonography for assessing the disease activity of sclerodermoid lupus erythematosus panniculitis.
Indian journal of dermatology, venereology and leprologyInvestigating the presence of neutrophil extracellular traps in septal and lobular cutaneous panniculitides.
International journal of dermatologyPatient and parent perspectives on transition from paediatric to adult healthcare in rheumatic diseases: an interview study.
BMJ openKikuchi-Fujimoto disease preceded by lupus erythematosus panniculitis: do these findings together herald the onset of systemic lupus erythematosus?
Dermatology online journalPoly-l-Lactic Acid for Treating Facial Lipoatrophy Secondary to Lupus Erythematosus Panniculitis.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Clinical Characteristics of Lupus Erythematosus Panniculitis/Profundus: A Retrospective Review of 61 Patients.
JAMA dermatologyUlcerative lupus erythematosus profundus in a patient with systemic lupus erythematosus and psoriasis.
The Journal of dermatologyIntralesional overlap syndrome: Sclerodermic lupus panniculitis and sclerodermic discoid lupus erythematosus.
JAAD case reportsTreatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab.
JAMA dermatologyIntravenous Immunoglobulin in the Management of Lupus Erythematosus Panniculitis.
Cureus[Clinical Comparative Analysis of Lupus Panniculitis and Subcutaneous Panniculitis-like T-cell Lymphoma].
Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science editionThe Challenge of Identifying Identical Twins: Advances in Differentiating Lupus Erythematosus Panniculitis from Subcutaneous Panniculitis-Like T-Cell Lymphoma.
SkinmedDiffuse multibacillary leprosy patient with Lucio's phenomenon and positive anticardiolipin antibody misdiagnosed as lupus erythematosus panniculitis in the People's Republic of China.
Infection and drug resistanceSubcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature.
The American Journal of dermatopathologyUtility of CD123 immunohistochemistry in differentiating lupus erythematosus from cutaneous T cell lymphoma.
HistopathologyDistinctive lupus panniculitis of scalp with linear alopecia along Blaschko's lines: a review of the literature.
International journal of dermatologyThe coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis-like T-cell lymphoma in the same patient.
JAAD case reportsSubcutaneous Panniculitis-Like T-Cell Lymphoma Versus Lupus Erythematosus Panniculitis: Distinction by Means of the Periadipocytic Cell Proliferation Index.
The American Journal of dermatopathologyGeometric alopecia associated with lupus erythematosus panniculitis of the scalp: a case series of nine Korean patients.
European journal of dermatology : EJDLupus erythematosus panniculitis in a 10-year-old female child with severe systemic lupus erythematosus: A case report.
MedicineOrbital T-cell lymphoma versus lupus panniculitits: a T-cell-mediated spectrum of orbital lymphoproliferative disease.
Orbit (Amsterdam, Netherlands)Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis.
Journal of cutaneous pathologyLupus erythematosus panniculitis resistant to standard treatment, complicated with macrophage activation syndrome.
Postepy dermatologii i alergologiiAnti-tumour necrosis factor α-induced lupus erythematosus panniculitis.
Journal of the European Academy of Dermatology and Venereology : JEADVCase of lupus erythematosus panniculitis triggered by human papillomavirus quadrivalent vaccine injection.
The Journal of dermatologyLinear Sclerodermoid Lupus Erythematosus Profundus in a Child.
The American Journal of dermatopathologyA Young Woman with Panniculitis and Cytopenia who Later Developed Coagulopathy.
The Journal of the Association of Physicians of IndiaEvaluation of Repairing Facial Depression Deformities Secondary to Lupus Erythematosus Panniculitis With Autologous Fat Grafting.
The Journal of craniofacial surgeryLupus Erythematosus Panniculitis in Pregnancy and Antimalarials.
Indian journal of dermatologyLupus Panniculitis as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report.
MedicineKikuchi-Fujimoto Disease Associated With Subcutaneous Panniculitis-Like T-Cell Lymphoma.
The American Journal of dermatopathologyUseful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis.
The American journal of surgical pathologyExtensive Lipoatrophy on a Buttock Revealing Systemic Lupus Erythematosus Panniculitis.
The Journal of rheumatologyLupus Erythematosus Panniculitis in Pregnancy.
Indian journal of dermatologyAssociações
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
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 um tipo de lúpus eritematoso crônico que se destaca por causar nódulos profundos na derme ou no tecido subcutâneo. As regiões mais acometidas pelas lesões costumam ser a cabeça, o rosto e os braços.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
4 fontes citadas no texto · 7 publicações do grafo RarasNet (PubMed) · 4 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 paniculite. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/lupus-eritematoso-paniculite
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Lúpus eritematoso paniculite
📋 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
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Reposicionamento
- fonte: Drug Repurposing Hub
