Raras
Buscar doenças, sintomas, genes...
Linfoma cutâneo primário
ORPHA:542DOENÇA RARA

Linfoma cutâneo é um grupo de doenças muito diverso, que se manifesta de várias formas. Ele varia bastante em suas características clínicas (o que o médico observa no paciente) e patológicas (o que se vê nos exames de laboratório), na forma como a doença evolui, no prognóstico (a previsão de como a doença vai se comportar), em sua causa molecular (ligada aos genes) e na resposta aos tratamentos. Todas essas particularidades foram consideradas nas classificações mais recentes. Nessas classificações, foi dada uma importância especial ao que cada um desses diferentes tipos significa para o prognóstico (o futuro da doença).

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Linfoma cutâneo é um grupo de doenças muito diverso, que se manifesta de várias formas. Ele varia bastante em suas características clínicas (o que o médico observa no paciente) e patológicas (o que se vê nos exames de laboratório), na forma como a doença evolui, no prognóstico (a previsão de como a doença vai se comportar), em sua causa molecular (ligada aos genes) e na resposta aos tratamentos. Todas essas particularidades foram consideradas nas classificações mais recentes. Nessas classificações, foi dada uma importância especial ao que cada um desses diferentes tipos significa para o prognóstico (o futuro da doença).

Pesquisas ativas
8 ensaios
32 total registrados no ClinicalTrials.gov
Publicações científicas
280 artigos
Último publicado: 2026 Mar
Medicamentos
5 registrados
BEXAROTENE, DENILEUKIN DIFTITOX, ROMIDEPSIN

Tem tratamento?

5 medicamentos registrados
Ver detalhes, fases e interações →
BEXAROTENEDENILEUKIN DIFTITOXROMIDEPSINVORINOSTATMOGAMULIZUMAB

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
🏥
SUS: Sem cobertura SUSScore: 0%
Você se identifica com essa condição?
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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

Partes do corpo afetadas

🧬
Pele e cabelo
17 sintomas
🩸
Sangue
8 sintomas
🫃
Digestivo
3 sintomas
🛡️
Imunológico
3 sintomas
🧠
Neurológico
2 sintomas
🫘
Rins
2 sintomas

+ 28 sintomas em outras categorias

Características mais comuns

Tremor
Placa eritematosa
Hepatoesplenomegalia
Pápula eritematosa
Anormalidade da cascata de coagulação
Fadiga
69sintomas
Sem dados (69)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 69 características clínicas mais associadas, ordenadas por frequência.

Tremor
Placa eritematosaErythematous plaque
HepatoesplenomegaliaHepatosplenomegaly
Pápula eritematosaErythematous papule
Anormalidade da cascata de coagulaçãoAbnormality of the coagulation cascade

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico280PubMed
Últimos 10 anos153publicações
Pico202021 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro ensaio clínico📈 2020Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

6 genes identificados com associação a esta condição.

HAVCR2Hepatitis A virus cellular receptor 2Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Cell surface receptor implicated in modulating innate and adaptive immune responses. Generally accepted to have an inhibiting function. Reports on stimulating functions suggest that the activity may be influenced by the cellular context and/or the respective ligand (PubMed:24825777). Regulates macrophage activation (PubMed:11823861). Inhibits T-helper type 1 lymphocyte (Th1)-mediated auto- and alloimmune responses and promotes immunological tolerance (PubMed:14556005). In CD8+ cells attenuates T

LOCALIZAÇÃO

Cell membraneCell junction

VIAS BIOLÓGICAS (1)
Interleukin-2 family signaling
EXPRESSÃO TECIDUAL(Ubíquo)
Pulmão
25.9 TPM
Baço
24.8 TPM
Sangue
18.0 TPM
Brain Spinal cord cervical c-1
14.4 TPM
Rim - Córtex
11.5 TPM
OUTRAS DOENÇAS (1)
subcutaneous panniculitis-like T-cell lymphoma
HGNC:18437UniProt:Q8TDQ0
TNFRSF1BTumor necrosis factor receptor superfamily member 1BCandidate gene tested inModerado
FUNÇÃO

Receptor with high affinity for TNFSF2/TNF and approximately 5-fold lower affinity for homotrimeric TNFSF1/lymphotoxin-alpha. The TRAF1/TRAF2 complex recruits the apoptotic suppressors BIRC2 and BIRC3 to TNFRSF1B/TNFR2. This receptor mediates most of the metabolic effects of TNF. Isoform 2 blocks TNF-induced apoptosis, which suggests that it regulates TNF function by antagonizing its biological activity

LOCALIZAÇÃO

Cell membraneSecreted

VIAS BIOLÓGICAS (4)
Neutrophil degranulationTNFR2 non-canonical NF-kB pathwayTNFs bind their physiological receptorsInterleukin-4 and Interleukin-13 signaling
EXPRESSÃO TECIDUAL(Ubíquo)
Sangue
474.3 TPM
Baço
180.8 TPM
Tecido adiposo
110.3 TPM
Linfócitos
105.9 TPM
Pulmão
100.4 TPM
OUTRAS DOENÇAS (2)
mycosis fungoidesSezary syndrome
HGNC:11917UniProt:P20333
CD28T-cell-specific surface glycoprotein CD28Candidate gene tested inRestrito
FUNÇÃO

Receptor that plays a role in T-cell activation, proliferation, survival and the maintenance of immune homeostasis (PubMed:1650475, PubMed:7568038). Functions not only as an amplifier of TCR signals but delivers unique signals that control intracellular biochemical events that alter the gene expression program of T-cells (PubMed:24665965). Stimulation upon engagement of its cognate ligands CD80 or CD86 increases proliferation and expression of various cytokines in particular IL2 production in bo

LOCALIZAÇÃO

Cell membraneCell surface

VIAS BIOLÓGICAS (2)
Co-stimulation by CD28Nef mediated downregulation of CD28 cell surface expression
MECANISMO DE DOENÇA

Immunodeficiency 123 with HPV-related verrucosis

An autosomal recessive immunologic disorder characterized by susceptibility to human papilloma virus (HPV) infections and the development of HPV-related common verrucosis in the first decade of life. In some patients with HPV2 infection, warts may progress to severe generalized hyperkeratotic cutaneous papillomatosis with cutaneous horns ('tree-man' phenotype). In patients with HPV4 infection, warts remains stable and may even regress with age.

OUTRAS DOENÇAS (3)
immunodeficiency 123 with HPV-related verrucosismycosis fungoidesSezary syndrome
HGNC:1653UniProt:P10747
CTLA4Cytotoxic T-lymphocyte protein 4Candidate gene tested inAltamente restrito
FUNÇÃO

Inhibitory receptor acting as a major negative regulator of T-cell responses (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:18641304, PubMed:28484017). Acts as a decoy receptor: the affinity of CTLA4 for its natural B7 family ligands, CD80 and CD86, is considerably stronger than the affinity of their cognate stimulatory coreceptor CD28 (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:28484017)

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (3)
Co-stimulation by CD28Co-inhibition by CTLA4RUNX1 and FOXP3 control the development of regulatory T lymphocytes (Tregs)
MECANISMO DE DOENÇA

Systemic lupus erythematosus

A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.

EXPRESSÃO TECIDUAL(Tecido-específico)
Baço
5.8 TPM
Intestino delgado
4.9 TPM
Pulmão
4.3 TPM
Testículo
2.5 TPM
Sangue
1.8 TPM
OUTRAS DOENÇAS (6)
autoimmune lymphoproliferative syndrome due to CTLA4 haploinsufficiencysystemic lupus erythematosusgranulomatosis with polyangiitismycosis fungoides
HGNC:2505UniProt:P16410
NPM1NucleophosminCandidate gene tested inAltamente restrito
FUNÇÃO

Involved in diverse cellular processes such as ribosome biogenesis, centrosome duplication, protein chaperoning, histone assembly, cell proliferation, and regulation of tumor suppressors p53/TP53 and ARF. Binds ribosome presumably to drive ribosome nuclear export. Associated with nucleolar ribonucleoprotein structures and bind single-stranded nucleic acids. Acts as a chaperonin for the core histones H3, H2B and H4. Stimulates APEX1 endonuclease activity on apurinic/apyrimidinic (AP) double-stran

LOCALIZAÇÃO

Nucleus, nucleolusNucleus, nucleoplasmCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (4)
PKR-mediated signalingTP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertainNuclear import of Rev proteinNuclear events stimulated by ALK signaling in cancer
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
1517.5 TPM
Fibroblastos
1222.0 TPM
Ovário
1031.3 TPM
Cervix Endocervix
640.5 TPM
Útero
622.0 TPM
OUTRAS DOENÇAS (8)
acute myeloid leukemiaacute promyelocytic leukemiaacute myeloid leukemia with NPM1 somatic mutationsdyskeratosis congenita
HGNC:7910UniProt:P06748
TYK2Non-receptor tyrosine-protein kinase TYK2Candidate gene tested inTolerante
FUNÇÃO

Tyrosine kinase of the non-receptor type involved in numerous cytokines and interferons signaling, which regulates cell growth, development, cell migration, innate and adaptive immunity (PubMed:10542297, PubMed:10995743, PubMed:7657660, PubMed:7813427, PubMed:8232552). Plays both structural and catalytic roles in numerous interleukins and interferons (IFN-alpha/beta) signaling (PubMed:10542297). Associates with heterodimeric cytokine receptor complexes and activates STAT family members including

LOCALIZAÇÃO

VIAS BIOLÓGICAS (10)
Interferon alpha/beta signalingRegulation of IFNA/IFNB signalingPotential therapeutics for SARSInterleukin-20 family signalingOther interleukin signaling
MECANISMO DE DOENÇA

Immunodeficiency 35

A primary immunodeficiency characterized by recurrent skin abscesses, pneumonia, and highly elevated serum IgE.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
100.5 TPM
Baço
95.0 TPM
Sangue
78.6 TPM
Pulmão
65.9 TPM
Nervo tibial
65.4 TPM
OUTRAS DOENÇAS (3)
immunodeficiency 35primary cutaneous anaplastic large cell lymphomalymphomatoid papulosis
HGNC:12440UniProt:P29597

Medicamentos e terapias

BEXAROTENEPhase 4

Mecanismo: Retinoid X receptor agonist

DENILEUKIN DIFTITOXPhase 4

Mecanismo: Interleukin-2 receptor binding agent

ROMIDEPSINPhase 4

Mecanismo: Histone deacetylase inhibitor

VORINOSTATPhase 4

Mecanismo: Histone deacetylase 6 inhibitor

MOGAMULIZUMABPhase 4

Mecanismo: C-C chemokine receptor type 4 cross-linking agent

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

88 variantes patogênicas registradas no ClinVar.

🧬 HAVCR2: GRCh37/hg19 5q33.2-34(chr5:154849998-164037131)x1 ()
🧬 HAVCR2: NM_032782.5(HAVCR2):c.833A>G (p.Asn278Ser) ()
🧬 HAVCR2: NM_032782.5(HAVCR2):c.319A>T (p.Ile107Phe) ()
🧬 HAVCR2: NC_000005.9:g.(?_155338082)_(156899968_?)del ()
🧬 HAVCR2: GRCh37/hg19 5q33.1-35.2(chr5:150535183-172906793)x3 ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado5
2Fase 26
1Fase 15
·Pré-clínico9
Medicamentos catalogadosEnsaios clínicos· 5 medicamentos · 20 ensaios
✓ Aprovados — podem ser usados hoje
BEXAROTENEDENILEUKIN DIFTITOXROMIDEPSINVORINOSTATMOGAMULIZUMAB
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Linfoma cutâneo primário

🗺️

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

🟢 Recrutando agora

4 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

32 ensaios clínicos encontrados, 8 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
157 papers (10 anos)
#1

Primary Cutaneous Follicle Center Lymphoma of the Nasal Dorsum and Tip Treated by Surgical Excision and Full-Thickness Skin Graft Reconstruction: A Case Report.

Cureus2026 Jan

Primary cutaneous follicle center lymphoma (PCFCL) is an indolent subtype of primary cutaneous B-cell lymphoma that may present diagnostic challenges due to its variable clinical appearance and frequent overlap with benign inflammatory or neoplastic skin conditions. We report the case of a 79-year-old male patient who presented with a persistent pruritic and erythematous lesion involving the nasal dorsum and tip. Initial dermatologic evaluation and limited skin biopsy failed to establish a definitive diagnosis, and the lesion was managed conservatively without improvement. Given persistent symptoms and anatomical involvement, wide surgical excision with oncologic intent was performed after systemic staging excluded extracutaneous disease. Immediate reconstruction was achieved using a full-thickness skin graft harvested from an upper blepharoplasty performed in the same operative session. Histopathological examination of the excised specimen confirmed the diagnosis of PCFCL with tumor-free margins. Postoperative recovery was uneventful, and at 12-month follow-up, the patient demonstrated satisfactory functional and aesthetic outcomes with no evidence of local recurrence. This case highlights the potential for PCFCL to masquerade as a benign inflammatory condition, the limitations of small diagnostic biopsies, and the importance of adequate tissue sampling. It also underscores the role of surgical excision with tailored reconstruction as an effective diagnostic and therapeutic strategy in selected cases of localized PCFCL involving the nasal region.

#2

Primary Cutaneous Lymphomas in Children: A Case Series Review with Emphasis on Diagnostic Imaging and Multidisciplinary Management.

Current medical imaging2026 Feb 02

Primary Cutaneous Lymphomas (PCLs) are rare extranodal non-Hodgkin lymphomas that present in the skin without extracutaneous disease at diagnosis; they are exceptionally uncommon in children and frequently mimic benign dermatoses, delaying recognition. We report a case series of three pediatric patients managed at a national referral center. Case 1: A 16-year-old with erythrodermic mycosis fungoides (stage IIIA) refractory to multiple systemic and skin-directed therapies who achieved remission after haploidentical allogeneic hematopoietic stem-cell transplantation. Case 2: A 3-year-old with aggressive cytotoxic CD8-positive Tcell lymphoma with EBV-associated disease and severe infectious complications during CHOP-based therapy, culminating in death despite salvage treatments and EBRT. Case 3: A 10-year-old with CD30-positive primary cutaneous anaplastic large cell lymphoma with a relapsing course, treated with BFM-90 chemotherapy, gemcitabine, pegylated doxorubicin plus total skin electron beam therapy, and ongoing PUVA with good dermatologic control. Across cases, diagnosis relied on clinicopathologic correlation with immunohistochemistry and staging CT; serum IgE was elevated in all three children. Pediatric PCLs show heterogeneous behavior and therapeutic responses. Early biopsy of atypical or treatment-refractory eruptions, comprehensive histopathology and immunophenotyping, targeted EBV testing when suggested, and appropriate use of skin-directed radiotherapy (EBRT/TSEBT) and transplantation in selected refractory disease are essential. Multidisciplinary management and equitable access to specialized therapies are critical to optimize outcomes.

#3

Haematopoietic stem cell transplant in cutaneous T-cell lymphomas: A multicentre propensity-score matched study.

Journal of the European Academy of Dermatology and Venereology : JEADV2026 Jan

Advanced cutaneous T-cell lymphomas (CTCL) are rare, generally refractory to therapeutic options, and have a poor prognosis. Haematopoietic stem cell transplantation (HSCT), mainly allogeneic HSCT (allo-HSCT), is considered a potentially curative option in CTCL refractory to other therapies. However, around half of patients relapse, and allo-HSCT is associated with significant adverse events. The available evidence on the usefulness of HSCT in CTCL generally comes from isolated cases and case series with a limited number of patients. Our aim was to evaluate the outcome of patients undergoing HSCT for advanced primary CTCL in Spain in a real-world environment and to compare their survival with that of similar patients who did not receive HSCT. We performed a retrospective observational study nested within the Primary Cutaneous Lymphoma Registry (RELCP) of the Spanish Academy of Dermatology and Venereology, collecting data on all patients receiving HSCT. Then, we performed propensity score matching (PSM) to pair HSCT patients with non-HSCT patients, adjusting for diagnosis, highest disease stage and age at diagnosis. We then performed survival analysis by means of Cox regression. Of 2848 patients included in the RELCP, 51 patients underwent HSCT. Thirty-six patients (70.6%) achieved a complete response and seven patients (13.7%) partial response. Relapse was developed by 56.9% of patients, and 39.2% died (19.6% due to disease progression and 15.7% due to HSCT complications, mainly graft-versus-host disease (GVHD) and sepsis). Overall survival (OS) after HSCT at 5 years was 58.9%. No differences in OS were found between HSCT and non-HSCT groups. We did not observe a survival benefit among HSCT patients compared to non-HSCT patients within the RELCP cohort. This could be due to patients having received a mean of 6.3 lines of treatment before HSCT. Larger studies might help identify subgroups of patients who might benefit from HSCT.

#4

Clinical Warburg Effect in a primary cutaneous lymphoma.

Clinical hematology international2026
#5

[Recent Research Advances in Classification and Treatment of Primary Cutaneous Lymphoma--Review].

Zhongguo shi yan xue ye xue za zhi2025 Dec

Primary cutaneous lymphoma (PCL) is a group of heterogeneous diseases. Traditional chemotherapy methods often result in substantial toxic side effects due to their low tumor selectivity. Currently, individualized therapy based on the genetic characteristics and target molecules of tumors constitutes the main treatment strategy for managing PCL. In recent years, research on novel treatment options has developed rapidly. These innovative therapies, which primarily include monoclonal antibodies, immune checkpoint inhibitors, small-molecule inhibitors, and chimeric antigen receptor T-cell (CAR-T) therapies, not only expand the treatment options for patients but also significantly improve their survival outcomes. The objective of this article is to provide a comprehensive review of the latest classification system for PCL and the most recent research advancements in cutting-edge treatment strategies. 原发性皮肤淋巴瘤的分类及治疗最新研究进展. 原发性皮肤淋巴瘤(PCL)是一组异质性疾病。传统的化疗方法因其较低的肿瘤选择性常常伴随着显著的毒副作用。目前,基于肿瘤的遗传学特征和靶分子的个体化治疗构成了PCL的主要治疗策略,关于新型治疗方案的研究在近年来发展迅速。这些新颖疗法主要包括单克隆抗体、免疫检查点抑制剂、小分子抑制剂和嵌合抗原受体T细胞疗法等,不仅为患者提供了更多的治疗选择,而且显著延长了患者的生存期。本文旨在对PCL的最新分类以及当前最新治疗方案的研究进展作一综述。 .

Publicações recentes

Ver todas no PubMed

📚 EuropePMC85 artigos no totalmostrando 152

2026

Primary Cutaneous Follicle Center Lymphoma of the Nasal Dorsum and Tip Treated by Surgical Excision and Full-Thickness Skin Graft Reconstruction: A Case Report.

Cureus
2026

Primary Cutaneous Lymphomas in Children: A Case Series Review with Emphasis on Diagnostic Imaging and Multidisciplinary Management.

Current medical imaging
2025

[Recent Research Advances in Classification and Treatment of Primary Cutaneous Lymphoma--Review].

Zhongguo shi yan xue ye xue za zhi
2026

Clinical Warburg Effect in a primary cutaneous lymphoma.

Clinical hematology international
2025

Angiogenesis and lymphangiogenesis in primary cutaneous lymphomas: a narrative review.

Clinical and experimental medicine
2025

Facial Presentation of Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type: A Rare Case.

Cureus
2025

Modern approaches to radiotherapy in primary cutaneous lymphomas: insights and recommendations from the DEGRO dermato-oncology working group.

Strahlentherapie und Onkologie : Organ der Deutschen Rontgengesellschaft ... [et al]
2025

Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children: Two Case Reports.

Clinical, cosmetic and investigational dermatology
2025

T- and NK-cell lymphomas and leukemias: Insights from the 2023 Society for Hematopathology/European Association for Haematopathology Workshop.

American journal of clinical pathology
2025

Care and treatment for primary cutaneous lymphoma-associated ulcers: Two case reports.

SAGE open medical case reports
2025

Primary cutaneous lymphoma is a microsatellite stable tumor: An analysis of microsatellite instability.

Drug discoveries &amp; therapeutics
2025

Tiger-man Appearance in a Case of DLBCL.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2025

How to treat primary cutaneous B cell lymphoma - Results from a monocentric cohort study on 98 patients.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

A current view of the pathogenesis and treatment of primary cutaneous diffuse large B cell lymphoma - leg type.

Leukemia &amp; lymphoma
2025

A Rare Case of Primary Cutaneous CD4+ Small/Medium Size T-Cell Lymphoproliferative Disorder Responding to Rituximab.

Case reports in dermatology
2025

Advances in classification and treatment of primary cutaneous lymphomas.

Annals of hematology
2025

Updated Classification of Cutaneous Lymphoma.

Advances in anatomic pathology
2026

Haematopoietic stem cell transplant in cutaneous T-cell lymphomas: A multicentre propensity-score matched study.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

A Gastrointestinal Manifestation of Primary Cutaneous Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma: A Case Report.

Cureus
2024

Non-Invasive Imaging Including Line-Field Confocal Optical Coherence Tomography (LC-OCT) for Diagnosis of Cutaneous Lymphomas.

Cancers
2024

Radiotherapy in cutaneous lymphomas: Recommendations from the EORTC cutaneous lymphoma tumour group.

European journal of cancer (Oxford, England : 1990)
2024

Secondary lymphomas of the skin.

Dermatology reports
2024

Enigmatic Presentation of Primary Cutaneous Lymphoma in a Boy.

Plastic and reconstructive surgery. Global open
2024

Prevalence, clinical features, and survival outcome trends of 627 patients with primary cutaneous lymphoma over 29 years: a retrospective review from single tertiary center in Korea.

Scientific reports
2024

Navigating Diagnostic and Therapeutic Challenges in Primary Cutaneous Gamma/Delta T-Cell Lymphoma: A Case Study of Fatal Outcomes Within Two Months.

The American Journal of dermatopathology
2024

Preliminary Experience in Ultra-High Frequency Ultrasound Assessment of Cutaneous Primary Lymphomas: An Innovative Classification.

Cancers
2025

Primary cutaneous lymphoma in children and adolescents: An epidemiological descriptive study in a Greek population.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Sézary Syndrome in West Sweden: Exploring Epidemiology, Clinical Features, and Treatment Patterns in a Registry-Based Retrospective Analysis.

Cancers
2024

Primary cutaneous, epidermotropic mycosis fungoides-like presentation: critical appraisal and description of two novel cases, broadening the spectrum of ALK+ T-cell lymphoma.

Virchows Archiv : an international journal of pathology
2025

Paediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Varied presentations of primary cutaneous lymphoma: A case series from a tertiary care center in South India.

Indian journal of cancer
2024

An unusual presentation of subcutaneous panniculitis-like T-cell lymphoma: Extensive necrosis and hemophagocytic lymphohistiocytosis: A case report.

Clinical case reports
2024

Extensive Cutaneous-Mucosal and Muscular Involvement of Gamma/Delta Cutaneous T-Cell Lymphoma on 18 F-FDG PET/CT.

Clinical nuclear medicine
2024

Bridging the specialty gap: Update on primary cutaneous lymphomas.

Journal of the American Academy of Dermatology
2023

Characteristics of Primary Cutaneous Lymphoma in Italy: A Tertiary Care, Single-Center Study.

Current oncology (Toronto, Ont.)
2024

Subcutaneous panniculitis-like T-cell lymphoma: Morphological, immunophenotypical and clonality assessment in six cats.

Veterinary dermatology
2023

[Clinical Features and Prognostic Factors of Patients with Primary Cutaneous Lymphoma].

Zhongguo shi yan xue ye xue za zhi
2023

Epidemiology of cutaneous T-cell lymphomas: state of the art and a focus on the Italian Marche region.

European journal of dermatology : EJD
2023

Case report: Successful treatment of Chidamide in a refractory/recurrent SPTCL with ARID1A mutation on the basis of CHOP plus auto-HSCT.

Medicine
2023

CD5+ Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type, Presenting as an Asymptomatic Nodule.

Hematology reports
2023

Immediate Response to Brentuximab Vedotin in a Patient with Localized MF-LCT.

Case reports in dermatology
2023

Primary cutaneous lymphoma in a patient with mastocytosis: Is there an association?

JAAD case reports
2023

An Update on Recent Advances of Photodynamic Therapy for Primary Cutaneous Lymphomas.

Pharmaceutics
2023

Pediatric Primary Cutaneous Lymphoma in China: A Retrospective Study.

Dermatology (Basel, Switzerland)
2023

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Scan Findings in a Rare Case of Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2023

The role of 18F-FDG PET/CT in primary cutaneous lymphoma: an educational review.

Annals of nuclear medicine
2023

Primary Cutaneous B-Cell Lymphomas with Large Cell Morphology: A Practical Review.

International journal of molecular sciences
2023

[Translated article] Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years.

Actas dermo-sifiliograficas
2023

Mycosis Fungoides and Sézary Syndrome: Microenvironment and Cancer Progression.

Cancers
2024

Synchronous occurrence of primary cutaneous B-cell lymphoma and cutaneous Rosai-Dorfman disease in distinct lesions: A unique association.

Journal of cutaneous pathology
2022

Rapidly progressive tumor stage mycosis fungoides: A case report from Syria.

Annals of medicine and surgery (2012)
2022

Primary Cutaneous B-Cell Lymphoma Co-Existing with Mycosis Fungoides-A Case Report and Overview of the Literature.

Life (Basel, Switzerland)
2022

Subcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report.

Case reports in dermatology
2022

Surgical, Dermatological and Morphopathological Considerations in the Diagnosis and Treatment of Cutaneous Lymphoproliferative Tumors, Primary CD4.

Medicina (Kaunas, Lithuania)
2023

Recent advances in cutaneous lymphoma-implications for current and future classifications.

Virchows Archiv : an international journal of pathology
2022

Diagnostic Outcomes and Treatment Modalities in Patients with Mycosis Fungoides in West Sweden-A Retrospective Register-Based Study.

Cancers
2023

Brentuximab vedotin in the treatment of cutaneous T-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Primary cutaneous lymphoma patients seen at a referral dermatological centre in 1 year: A single-centre observational retrospective cohort study of the diagnoses and staging, comorbidities and associated symptoms, treatment performed and clinical course.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Role of PET imaging in peritoneal involvement of subcutaneous panniculitis-like T-cell lymphoma.

Journal of radiology case reports
2022

Primary cutaneous lymphoma: the 2018 update of the WHO-EORTC classification.

Presse medicale (Paris, France : 1983)
2022

Primary cutaneous lymphoma and risk for severe COVID-19: a prospective study of 48 cases in Morocco.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

A Case of CD5-Positive Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type Secondary to Chronic Lymphedema.

The American Journal of dermatopathology
2022

Pediatric Cutaneous Hematologic Disorders: Cutaneous Lymphoma and Leukemia Cutis-Experience of a Tertiary-Care Pediatric Institution and Review of the Literature.

Journal of cutaneous medicine and surgery
2021

Safety and danger of biologic treatments in psoriasis in context of cutaneous T-cell lymphoma (CTCL).

Postepy dermatologii i alergologii
2022

Primary cutaneous gamma/delta T-cell lymphoma in Taiwan: A series of six cases with frequent solitary presentation and relatively indolent behavior.

Journal of cutaneous pathology
2022

Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC.

Blood
2021

Primary cutaneous lymphomas in children: A prospective study from the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry.

Pediatric dermatology
2021

[New national treatment guidelines for primary cutaneous lymphoma].

Lakartidningen
2021

Primary cutaneous indolent B-cell lymphomas - a retrospective multicenter analysis and a review of literature.

Acta oncologica (Stockholm, Sweden)
2021

Report of Eleven Patients of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Clinicopathologic Features, 18F-FDG PET/CT Findings and Outcome.

Frontiers in oncology
2021

Patients with primary cutaneous lymphoma are at risk for severe COVID-19. Data from the Spanish Primary Cutaneous Lymphoma Registry.

Journal of the European Academy of Dermatology and Venereology : JEADV
2021

Single-cell RNA sequencing profiling in a patient with discordant primary cutaneous B-cell and T-cell lymphoma reveals micromilieu-driven immune skewing.

The British journal of dermatology
2021

A Case of Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm Treated With a Bcl-2 Inhibitor.

Journal of drugs in dermatology : JDD
2021

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review.

International journal of immunopathology and pharmacology
2021

Primary cutaneous lymphoma: a population-based cancer registry descriptive study of 155 consecutive cases diagnosed between 1979 and 2016.

Leukemia &amp; lymphoma
2021

Primary Cutaneous Acral CD8+ T-cell Lymphoma Induced by Persistent Delayed Hypersensitivity to Gold Earrings.

Actas dermo-sifiliograficas
2021

Mycosis Fungoides with Dermal Mucin Deposition: An Unusual Variant of the Most Challenging Primary Cutaneous Lymphoma.

Case reports in dermatology
2021

Helical Skin Radiation Therapy Including Total Skin Radiation Therapy Using Tomotherapy for Primary Cutaneous Lymphoma With Bone Marrow Suppression as a Related Adverse Event.

Practical radiation oncology
2020

Corrigendum: Management of primary cutaneous lymphoma patients during COVID-19 pandemic: EORTC CLTF guidelines.

Journal of the European Academy of Dermatology and Venereology : JEADV
2021

The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series.

Dermatology (Basel, Switzerland)
2020

Primary cutaneous peripheral T-cell lymphoma, not otherwise specified with mammalian target of rapamycin mutation: A novel finding for targeted treatment.

JAAD case reports
2021

Acral lymphomatoid papulosis: Report of five cases, differential diagnosis, and review.

Journal of cutaneous pathology
2021

Infliximab-related atypical lymphoid hyperplasia mimicking primary cutaneous lymphoma of the ear.

Dermatologic therapy
2020

Dermoscopy of Syringotropic and Folliculotropic Mycosis Fungoides.

Dermatology practical &amp; conceptual
2021

Cutaneous Lymphomas -Part II: Other Cutaneous Lymphomas.

Actas dermo-sifiliograficas
2020

Borrelia burgdorferi in primary cutaneous lymphomas: a systematic review and meta-analysis.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2021

Cutaneous Lymphomas - Part I: Mycosis Fungoides, Sézary Syndrome, and CD30+ Cutaneous Lymphoproliferative Disorders.

Actas dermo-sifiliograficas
2020

Clinical Remission in a 72-Year-Old Patient with a Massive Primary Cutaneous Peripheral T-Cell Lymphoma-NOS of the Eyelid, Following Combination Chemotherapy with Etoposide Plus COP.

Diagnostics (Basel, Switzerland)
2020

Time to Next Treatment as a Meaningful Endpoint for Trials of Primary Cutaneous Lymphoma.

Cancers
2020

Systemic rituximab for the treatment of the indolent forms of primary cutaneous B-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

Journal of the American Academy of Dermatology
2021

A case of primary cutaneous B-cell lymphoma with immature features in an old man. Diffuse large B-cell lymphoma with immature features or B-cell lymphoblastic lymphoma?

Journal of cutaneous pathology
2020

Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type: A Case Report.

Cureus
2020

Total skin electron beam therapy for primary cutaneous T-cell lymphomas: clinical characteristics and outcomes in a Mexican reference center.

Reports of practical oncology and radiotherapy : journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation Oncology
2021

A Case Report of Pityriasis Lichenoides-Like Mycosis Fungoides in Children: A Challenging Diagnosis.

Korean journal of family medicine
2020

Management of primary cutaneous lymphoma patients during COVID-19 pandemic: EORTC CLTF guidelines.

Journal of the European Academy of Dermatology and Venereology : JEADV
2021

The prognostic role of age in primary cutaneous B-cell lymphoma: a proposal derived from a population-based registry.

Aging clinical and experimental research
2020

Diffuse large B cell lymphoma progression with skin involvement: A case report.

Journal of tissue viability
2020

Osteoma cutis in the setting of primary cutaneous lymphoma.

Journal of cutaneous pathology
2020

18FDG PET/CT in Primary Cutaneous Diffuse Large B-Cell Lymphoma Leg Type.

Clinical nuclear medicine
2020

Mycosis Fungoides: A Clinicopathological Study of 60 Cases from a Tertiary Care Center.

Indian journal of dermatology
2020

Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Long-Term Indolent Clinical Course Initially Mimicking Lupus Erythematosus Profundus.

Frontiers in oncology
2020

Primary cutaneous γδ T-cell lymphoma with unusual immunophenotype: A case report and review of published work.

The Journal of dermatology
2020

The clinico-pathological spectrum of primary cutaneous lymphoma other than mycosis fungoides/Sezary syndrome.

Virchows Archiv : an international journal of pathology
2020

Molecular profiling of TOX-deficient neoplastic cells in cutaneous T cell lymphoma.

Archives of dermatological research
2020

A retrospective review of cutaneous lymphoma in Botswana.

International journal of dermatology
2020

Mycosis fungoides in pediatric patients: Clinical features, diagnostic challenges, and advances in therapeutic management.

Pediatric dermatology
2020

Associated primary tumours in patients with primary cutaneous lymphoma: a single-centre retrospective cohort study.

Journal of the European Academy of Dermatology and Venereology : JEADV
2019

Recent Advances in Cutaneous T-cell Lymphoma: Diagnostic and Prognostic Considerations.

Surgical pathology clinics
2019

Clinical characteristics of primary cutaneous lymphoma: analysis from two centres in China.

The British journal of dermatology
2019

Treatment of primary cutaneous anaplastic large cell lymphoma.

Archives of craniofacial surgery
2019

RF - Brentuximab as Treatment for CD30+ Primary Cutaneous Lymphoma.

Actas dermo-sifiliograficas
2019

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos.

Gaceta medica de Mexico
2019

Subcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature.

The American Journal of dermatopathology
2019

Recent advances in localized immunotherapy of skin cancers.

Immunotherapy
2018

Primary cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma: a rare and aggressive cutaneous lymphoma.

Cutis
2019

The clinical value of imaging in primary cutaneous lymphomas: Role of high resolution ultrasound and PET-CT.

The British journal of radiology
2018

Early dermoscopic sign of folliculotropism in patients with mycosis fungoides.

Dermatology practical &amp; conceptual
2019

Primary cutaneous lymphoma in Argentina: a report of a nationwide study of 416 patients.

International journal of dermatology
2019

Local radiation for cutaneous T-cell lymphoma other than mycosis fungoides and Sézary syndrome.

Chinese clinical oncology
2018

Light and shadows of a new technique: is photon total-skin irradiation using helical IMRT feasible, less complex and as toxic as the electrons one?

Radiation oncology (London, England)
2018

Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients.

Journal of the American Academy of Dermatology
2018

Clinical and epidemiological profile of patients with early stage mycosis fungoides.

Anais brasileiros de dermatologia
2018

Hydromorphone-induced chorea as an atypical presentation of opioid neurotoxicity: A case report and review of the literature.

Palliative medicine
2018

[Primary cutaneous lymphoma-a case series of 163 patients].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
2018

The First Year of the AEVD Primary Cutaneous Lymphoma Registry.

Actas dermo-sifiliograficas
2018

Primary cutaneous lymphoma in children: A report of four cases.

The Turkish journal of pediatrics
2018

Mycosis Fungoides Manifesting as Giant Cell Lichenoid Dermatitis.

The American Journal of dermatopathology
2017

[Diagnostics of primary cutaneous lymphomas].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
2017

Brentuximab vedotin in CD30+ primary cutaneous T-cell lymphomas: a review and analysis of existing data.

International journal of dermatology
2017

Cutaneous Lymphomas with Cytotoxic Phenotype.

Surgical pathology clinics
2017

Renaissance of Low-Dose Radiotherapy Concepts for Cutaneous Lymphomas.

Oncology research and treatment
2017

AEDV Primary Cutaneous Lymphoma Registry.

Actas dermo-sifiliograficas
2017

A 92-year-old man with primary cutaneous diffuse large B-cell non-Hodgkin's lymphoma manifesting as a giant scalp mass: A case report.

Medicine
2018

Primary cutaneous non-Hodgkin lymphoma: results of a retrospective analysis in the light of the recent ILROG guidelines.

Tumori
2017

Aggressive cutaneous T-cell lymphomas.

Seminars in diagnostic pathology
2017

The role of 18F-FDG PET and PET/CT in the evaluation of primary cutaneous lymphoma.

Nuclear medicine communications
2016

Paraneoplastic Dermatosis in a Patient with Anaplastic Large-Cell Lymphoma: Case Report and Literature Review.

Dermatopathology (Basel, Switzerland)
2016

Mechanism, Consequences, and Therapeutic Targeting of Abnormal IL15 Signaling in Cutaneous T-cell Lymphoma.

Cancer discovery
2016

Importance of assessing nonattenuation-corrected positron emission tomography images in treatment response evaluation of primary cutaneous lymphoma.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2016

Diagnosing Spindle Cell Variant of Primary Cutaneous B-Cell Lymphoma: Potential Pitfalls and Solutions.

Annals of clinical and laboratory science
2016

Adolescent weight and height are predictors of specific non-Hodgkin lymphoma subtypes among a cohort of 2,352,988 individuals aged 16 to 19 years.

Cancer
2015

Primary cutaneous lymphomas: diagnosis and treatment.

Postepy dermatologii i alergologii
2015

Primary Cutaneous Lymphoma-Associated Pseudoepitheliomatous Hyperplasia Masquerading as Squamous Cell Carcinoma in a Young Adult.

Acta medica Iranica
2015

Primary cutaneous lymphoma in southern Brazil: a 12-year single-center experience.

International journal of dermatology
2015

Diagnosis and Management of Cutaneous B-cell Lymphoma.

Dermatologic clinics
2015

Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma.

Dermatologic clinics
2016

Illness Perception in Primary Cutaneous T-cell Lymphomas: What Patients Believe About Their Disease.

Acta dermato-venereologica
2015

Galectin-3 Expression in Primary Cutaneous CD30-Positive Lymphoproliferative Disorders and Transformed Mycosis Fungoides.

Dermatology (Basel, Switzerland)
2015

Primary cutaneous natural killer/T-cell lymphoma of the nasal type: a report of 4 cases in North American patients.

Annals of diagnostic pathology
2015

Sixty years old man with primary cutaneous lymphoma.

Mymensingh medical journal : MMJ
2015

Best practices in diagnostic immunohistochemistry: workup of cutaneous lymphoid lesions in the diagnosis of primary cutaneous lymphoma.

Archives of pathology &amp; laboratory medicine
2015

Frequency of primary cutaneous lymphoma variants in Austria: retrospective data from a dermatology referral centre between 2006 and 2013.

Journal of the European Academy of Dermatology and Venereology : JEADV

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Doenças relacionadas

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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.

  1. Primary Cutaneous Follicle Center Lymphoma of the Nasal Dorsum and Tip Treated by Surgical Excision and Full-Thickness Skin Graft Reconstruction: A Case Report.
    Cureus· 2026· PMID 41728511mais citado
  2. Primary Cutaneous Lymphomas in Children: A Case Series Review with Emphasis on Diagnostic Imaging and Multidisciplinary Management.
    Current medical imaging· 2026· PMID 41691664mais citado
  3. Haematopoietic stem cell transplant in cutaneous T-cell lymphomas: A multicentre propensity-score matched study.
    Journal of the European Academy of Dermatology and Venereology : JEADV· 2026· PMID 40065681mais citado
  4. Clinical Warburg Effect in a primary cutaneous lymphoma.
    Clinical hematology international· 2026· PMID 41492311mais citado
  5. [Recent Research Advances in Classification and Treatment of Primary Cutaneous Lymphoma--Review].
    Zhongguo shi yan xue ye xue za zhi· 2025· PMID 41502290mais citado
  6. Moderate-Dose Local Radiotherapy in Symptomatic CD30-Positive, Anaplastic Lymphoma Kinase (ALK)-Negative Primary Cutaneous T-cell Lymphoma.
    Cureus· 2026· PMID 41970102recente
  7. Acalabrutinib treatment in relapsed/refractory primary cutaneous diffuse large B cell lymphoma, leg type. Case report.
    Front Oncol· 2026· PMID 41919263recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:542(Orphanet)
  2. MONDO:0018898(MONDO)
  3. GARD:18698(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q1794643(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

Linfoma cutâneo primário
Compêndio · Raras BR

Linfoma cutâneo primário

ORPHA:542 · MONDO:0018898
Prevalência
1-9 / 100 000
Ensaios
8 ativos
Medicamentos
5 registrados
Prevalência
0.0 (Europe)
MedGen
UMLS
C1276146
EuropePMC
Wikidata
Papers 10a
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