O lúpus eritematoso túmido é considerado um tipo raro de lúpus eritematoso cutâneo crônico. O lúpus eritematoso cutâneo (LEC) pode ser dividido em lúpus cutâneo agudo, lúpus cutâneo subagudo e lúpus cutâneo crônico. O lúpus eritematoso túmido é caracterizado por espinhas (pápulas) lisas, sem cicatrizes, de cor rosa a violeta, sem quaisquer outras alterações cutâneas aparentes, como cicatrizes. Pacientes com lúpus eritematoso túmido geralmente não apresentam outros sintomas de lúpus eritematoso sistêmico ou outros tipos de lúpus eritematoso cutâneo. As pápulas aparecem nas áreas expostas ao sol da face, parte superior das costas, região V do pescoço, tronco e braços e, mais raramente, nas coxas e pernas. Geralmente afetam igualmente ambos os lados do corpo, mas podem afetar apenas um lado. Normalmente, as pápulas desaparecem sem deixar cicatrizes. O tratamento é muito eficaz na maioria dos casos e pode incluir proteção solar, medicamentos antimaláricos, corticosteróides locais, tacrolimus tópico e fototerapia.
Introdução
O que você precisa saber de cara
O lúpus eritematoso túmido é considerado um tipo raro de lúpus eritematoso cutâneo crônico. O lúpus eritematoso cutâneo (LEC) pode ser dividido em lúpus cutâneo agudo, lúpus cutâneo subagudo e lúpus cutâneo crônico. O lúpus eritematoso túmido é caracterizado por espinhas (pápulas) lisas, sem cicatrizes, de cor rosa a violeta, sem quaisquer outras alterações cutâneas aparentes, como cicatrizes. Pacientes com lúpus eritematoso túmido geralmente não apresentam outros sintomas de lúpus eritematoso sistêmico ou outros tipos de lúpus eritematoso cutâneo. As pápulas aparecem nas áreas expostas ao sol da face, parte superior das costas, região V do pescoço, tronco e braços e, mais raramente, nas coxas e pernas. Geralmente afetam igualmente ambos os lados do corpo, mas podem afetar apenas um lado. Normalmente, as pápulas desaparecem sem deixar cicatrizes. O tratamento é muito eficaz na maioria dos casos e pode incluir proteção solar, medicamentos antimaláricos, corticosteróides locais, tacrolimus tópico e fototerapia.
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
Partes do corpo afetadas
+ 5 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 12 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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 túmido
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Pesquisa ativa
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1 ensaios clínicos encontrados.
Publicações mais relevantes
Rare Lupus Exacerbation Shines Light on Provider Bias in a Patient With Possible Autoimmune Hepatitis.
A 42-year-old woman with a past medical history of alcohol use disorder, decompensated alcoholic cirrhosis, systemic lupus erythematosus (SLE), rheumatoid arthritis, and celiac disease presented to the emergency department (ED) for a four-day history of painful rash with worsening pruritus and acute kidney injury. After admission, dermatology, rheumatology, and nephrology teams were consulted, and a skin and renal biopsy were obtained, with results significant for lupus erythematosus tumidus (LET), lupus nephritis grade III (not active), and IgA nephritis, consistent with a systemic lupus erythematosus exacerbation. Despite the patient's history of autoimmune disease, she had previously never received a biopsy for autoimmune hepatitis (AIH) during her initial diagnosis of cirrhosis. During her admission, an autoimmune hepatitis panel was obtained with results suggestive of autoimmune hepatitis; however, a conclusive diagnosis requires a liver biopsy, which was not obtained inpatient. In patients with a strong history of alcohol use, anchoring on substance use as the sole driver of liver disease may be easy, but this case highlights the importance of keeping a wide differential and considering related illnesses, even in patients with pre-existing diagnoses.
Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study.
Lupus erythematosus tumidus (LET) is a rare form of cutaneous lupus erythematosus (CLE) with a controversial connection to systemic lupus erythematosus (SLE). We conducted a retrospective study with 34 LET patients in a Spanish tertiary referral center from 2007 to 2019. Most were women [52.9% (18/34)], with a median age of 53.5 years. Autoimmune or rheumatologic disorders were reported in 52.9% (18/34) of cases, and other CLE variants in 26.5% (9/34). SLE occurred in 8.82% (3/34), while 64.7% (22/34) had autoantibodies. Immunohistochemical CD123 testing tested positive in 75.9% (22/34), while direct immunofluorescence (DIF) showed positivity in 31.8% (7/22) of cases. Treatment included topical agents (100%), antimalarials (73.5%), oral corticosteroids (23.5%), and immunosuppressants (14.7%). All achieved clinical remission, but a delayed response (>3 months) was linked to SLE (p=0.002) and anti-DNA antibodies (p=0.003). LET usually associates with autoimmune disorders and autoantibodies. CD123 and DIF aid diagnosis, and systemic treatment may be needed, especially with SLE and anti-DNA antibodies.
Abatacept-induced lupus erythematosus tumidus: A case report and literature review.
Off-label dermatologic uses of IL-23 inhibitors.
While IL-23 inhibitors, which include guselkumab, tildrakizumab, and risankizumab, are currently FDA-approved solely for the treatment of psoriasis, several other inflammatory skin conditions have been associated with elevated IL-23 levels. The purpose of this review is to summarize and interpret the literature surrounding the off-label uses of IL-23 inhibitors in dermatologic practice. We conducted searches on PubMed and ClinicalTrials.gov for clinical trials, observational studies, case series, and case reports assessing use of the three IL-23 inhibitors for non-psoriatic dermatologic conditions. Conditions exhibiting promising response to treatment with IL-23 inhibitors include hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris, pyoderma gangrenosum, dissecting cellulitis of the scalp, congenital ichthyosiform erythroderma, lichen planus pemphigoides, acrofacial vitiligo, lichen planopilaris, frontal fibrosing alopecia, lupus erythematosus tumidus, and Stewart-Treves angiosarcoma. Current literature suggests that IL-23 inhibitors may be effective in treating these conditions due to shared pathophysiologic pathways with psoriasis. Although these results are promising, further research through large-scale, randomized clinical trials is needed to further evaluate the efficacy and safety of IL-23 inhibitors in treating these off-label conditions.
Lupus Erythematosus Tumidus Misdiagnosed as Erythema Nodosum from Coccidioidomycosis.
Lupus erythematosus tumidus (LET) is a rare photosensitive dermatosis that is categorized as intermittent cutaneous lupus erythematosus. It shares clinical similarities and histopathological features with other skin disorders, such as erythema nodosum, lymphocytic infiltrate of Jessner, and reticular erythematous mucinosis, thus making diagnosis quite challenging. We present a patient with LET whose diagnosis was confirmed after seeing several doctors. A 52-year-old Hispanic female presented with tender erythematous nodules on her thighs for approximately 1 month. She was suspected of having erythema nodosum secondary to coccidioidomycosis and was prescribed fluconazole 200 mg for 30 days but showed no improvement. However, histopathological and direct immunofluorescence tests later confirmed a diagnosis of LET. The patient was treated with hydroxychloroquine, and the lesions improved remarkably after 2 weeks. LET is a rare dermatosis that closely resembles other dermatologic conditions such as erythema nodosum, lymphocytic infiltrate of Jessner, and reticular erythematous mucinosis. Diagnosis based on clinical features alone should be avoided, and ideally, treatment should only be initiated after confirmatory histopathological testing.
Publicações recentes
Lupus Erythematosus Tumidus Mimicking Erythema Nodosum: A Diagnostic Challenge.
Rare Lupus Exacerbation Shines Light on Provider Bias in a Patient With Possible Autoimmune Hepatitis.
Abatacept-induced lupus erythematosus tumidus: A case report and literature review.
Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study.
Off-label dermatologic uses of IL-23 inhibitors.
📚 EuropePMC78 artigos no totalmostrando 44
Rare Lupus Exacerbation Shines Light on Provider Bias in a Patient With Possible Autoimmune Hepatitis.
CureusAbatacept-induced lupus erythematosus tumidus: A case report and literature review.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGClinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study.
Actas dermo-sifiliograficasOff-label dermatologic uses of IL-23 inhibitors.
The Journal of dermatological treatmentLupus Erythematosus Tumidus Misdiagnosed as Erythema Nodosum from Coccidioidomycosis.
Case reports in dermatologyHydroxychloroquine-induced generalized myopathy in a patient with lupus tumidus: a case report.
Dermatology reportsLupus Erythematosus Tumidus as a Distinct Uncommon Subtype of Cutaneous Lupus Erythematosus: A Case Report and Review.
CureusTreatment of recalcitrant lupus erythematosus tumidus with deucravacitinib.
JAAD case reportsAtorvastatin-induced Lupus Erythematosus Tumidus: A Case Report and Literature Review.
Acta dermatovenerologica Croatica : ADCEfficacy of upadacitinib in a case of resistant lupus erythematosus tumidus.
Journal of the European Academy of Dermatology and Venereology : JEADVSuccessful treatment of a child's lupus erythematosus tumidus with antimalarials.
Postepy dermatologii i alergologiiSystemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report.
Journal of medical case reportsUnilateral periorbital lupus erythematosus tumidus mimicking heliotrope rash.
The Journal of dermatologyLupus Erythematosus Tumidus Clinical Characteristics and Treatment: A Retrospective Review of 25 Patients.
CutisDermoscopic Features of Acute, Subacute, Chronic and Intermittent Subtypes of Cutaneous Lupus Erythematosus in Caucasians.
Journal of clinical medicineLupus Erythematosus Tumidus: Clinical and Pathological Features in a Series of 20 Patients.
The American Journal of dermatopathologyAn unusual presentation of lupus erythematosus tumidus in a child.
International journal of dermatologyLupus Erythematosus Tumidus Associated With Hemophagocytosis.
The American Journal of dermatopathologyA teen with blaschkolinear tumid lupus erythematosus.
JAAD case reportsDevelopment of Lupus Erythematosus Tumidus During the Course of Systemic Sclerosis.
CureusLupus erythematosus tumidus in a patient with mycosis fungoides stage IB after complete response to PUVA.
Journal of the European Academy of Dermatology and Venereology : JEADVCase of hereditary angioedema with lupus erythematosus tumidus-like eruption.
The Australasian journal of dermatologyLupus erythematosus tumidus in an adolescent with beta thalassemia minor successfully controlled with hydroxychloroquine.
Dermatologic therapyHemifacial erythema: an unusual manifestation of lupus erythematosus tumidus.
Rheumatology (Oxford, England)[Reticular erythematous mucinosis-A special subtype of cutaneous lupus erythematosus?].
Zeitschrift fur RheumatologieLupus Erythematosus Tumidus Mimicking Primary Cutaneous Marginal Zone B-cell Lymphoma.
Acta dermato-venereologicaLupus Erythematosus Tumidus with Pseudolymphomatous Infiltrates: A Case Report.
Journal of Nippon Medical School = Nippon Ika Daigaku zasshiAn atypical clinical presentation of alopecia in 2 patients with systemic lupus erythematosus.
JAAD case reportsLupus erythematosus tumidus: clinical perspectives.
Clinical, cosmetic and investigational dermatologyA unique presentation of lupus erythematosus tumidus in an adolescent boy.
CutisRefractory lupus erythematosus tumidus responsive to tildrakizumab.
Dermatologic therapyPeriorbital swelling and episcleritis may be a sign of cutaneous lupus erythematosus.
Clinical case reportsHeterogeneous lupus-specific lesions and treatment outcome, in a single patient, over a period of time.
Clinical case reportsReticular Erythematous Mucinosis: A Rare Cutaneous.
Acta dermatovenerologica Croatica : ADCDisease severity and prophylactic measures in patients with cutaneous lupus erythematosus: results of a worldwide questionnaire-based study.
Postepy dermatologii i alergologiiA case report of lupus erythematosus tumidus converted from discoid lupus erythematosus.
MedicineLupus erythematosus tumidus: Benign disease in children?
Pediatrics international : official journal of the Japan Pediatric SocietyA case of a focal oedematous reaction induced by rituximab overlying lupus erythematosus tumidus.
European journal of dermatology : EJDRituximab in the treatment of recalcitrant generalized lupus erythematosus tumidus.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGAn unusual clinical presentation of lupus erythematosus tumidus localized on the thigh.
Clinical and experimental dermatologyLupus erythematosus tumidus of the scalp masquerading as alopecia areata.
CutisLupus erythematosus tumidus: A case and discussion of a rare entity in black patients.
JAAD case reportsMepacrine as successful monotherapy for refractory Jessner-Kanof disease: still an important drug in the dermatologic armamentarium.
The Journal of dermatological treatmentUstekinumab-induced drug eruption resembling lymphocytic infiltration (of Jessner-Kanof) and lupus erythematosus tumidus.
British journal of clinical pharmacologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Rare Lupus Exacerbation Shines Light on Provider Bias in a Patient With Possible Autoimmune Hepatitis.
- Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study.
- Abatacept-induced lupus erythematosus tumidus: A case report and literature review.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG· 2025· PMID 40342196mais citado
- Off-label dermatologic uses of IL-23 inhibitors.
- Lupus Erythematosus Tumidus Misdiagnosed as Erythema Nodosum from Coccidioidomycosis.
- Lupus Erythematosus Tumidus Mimicking Erythema Nodosum: A Diagnostic Challenge.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:90283(Orphanet)
- MONDO:0019560(MONDO)
- Lupus Eritematoso Sistemico(PCDT · Ministério da Saúde)
- GARD:13003(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q3267714(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
