A síndrome de Bazex-Dupre-Christol é uma doença de pele hereditária rara, que tem uma tendência a desenvolver carcinomas basocelulares (um tipo de câncer de pele) precocemente.
Introdução
O que você precisa saber de cara
A síndrome de Bazex-Dupre-Christol é uma doença de pele hereditária rara, que tem uma tendência a desenvolver carcinomas basocelulares (um tipo de câncer de pele) precocemente.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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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
+ 17 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 43 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 — Síndrome Bazex
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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
0 ensaios clínicos encontrados.
Publicações mais relevantes
Acrokeratosis Paraneoplastica (Bazex Syndrome)-Like Dermatomyositis With Anti-PM/Scl Antibodies.
The Importance and Challenges of Early Diagnosis of Paraneoplastic Skin Syndromes in Cancer Detection-A Review.
Skin paraneoplastic syndromes (SPNSs) are a group of disorders that arise as a consequence of cancer but are not directly related to the tumor mass itself. This review aims to provide a comprehensive overview of these syndromes, encompassing their pathophysiology, clinical features, diagnostic approaches, differential diagnosis, and management strategies. These syndromes, which include conditions such as Bazex syndrome, acanthosis nigricans, dermatomyositis, and necrolytic migratory erythema often manifest prior to or concurrently with a cancer diagnosis, serving as potential early warning signs of underlying malignancies. This review delves into the spectrum of SPNSs and their associations with specific cancer types. Special emphasis is placed on the critical role of dermatologists and oncologists in identifying these skin manifestations as potential markers of malignancy. By raising awareness of SPNSs, this paper highlights the pivotal importance of prompt recognition and intervention in reducing cancer-related mortality.
A rare case of acrokeratosis paraneoplastica (Bazex syndrome) and a literature review.
Acrokeratosis paraneoplastica is a rare and obligate paraneoplastic dermatosis most commonly associated with squamous cell carcinoma of the upper aerodigestive tract and lung. It has also been linked to adenocarcinomas of the lung, colon, and stomach. However, reports of its association with esophageal adenocarcinoma are exceedingly rare, with only one documented case to date. This condition primarily affects middle-aged White men, with limited cases reported in women. Moreover, its occurrence in the Chinese population is uncommon. We herein present a case of acrokeratosis paraneoplastica in a Chinese woman with primary esophageal adenocarcinoma, along with a review of the relevant literature. To our knowledge, this is the first documented instance of paraneoplastic acrokeratosis associated with esophageal adenocarcinoma in a woman.
Recurrence of acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of metastatic recurrence of squamous cell carcinoma of the lung.
[Palmoplantar dermatoses: Diagnostic approach in primary care].
This article addresses the diagnostic challenges of palmoplantar dermatoses (PPD) within the scope of Primary Health Care (PHC). These common skin conditions, encountered in daily practice, exhibit a diverse range of symptoms and morphologies, complicating their diagnosis. They are etiologically classified into infectious inflammatory, non-infectious inflammatory, and hereditary keratodermas. While various dermatoses may affect the palms and soles, few are specific to this area. Notable examples include palmoplantar pustulosis, dyshidrosis, erythema pernio, and Bazex syndrome. Given the high prevalence of dermatological consultations in PHC, this article underscores the significance of PHC professionals' knowledge regarding these conditions. It proposes a diagnostic algorithm to facilitate their management and timely referral.
Publicações recentes
Recurrence of acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of metastatic recurrence of squamous cell carcinoma of the lung.
Acrokeratosis Paraneoplastica (Bazex Syndrome)-Like Dermatomyositis With Anti-PM/Scl Antibodies.
The Importance and Challenges of Early Diagnosis of Paraneoplastic Skin Syndromes in Cancer Detection-A Review.
A rare case of acrokeratosis paraneoplastica (Bazex syndrome) and a literature review.
[Palmoplantar dermatoses: Diagnostic approach in primary care].
📚 EuropePMC98 artigos no totalmostrando 45
Recurrence of acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of metastatic recurrence of squamous cell carcinoma of the lung.
Dermatology online journalAcrokeratosis Paraneoplastica (Bazex Syndrome)-Like Dermatomyositis With Anti-PM/Scl Antibodies.
The Journal of dermatologyThe Importance and Challenges of Early Diagnosis of Paraneoplastic Skin Syndromes in Cancer Detection-A Review.
CancersA rare case of acrokeratosis paraneoplastica (Bazex syndrome) and a literature review.
The Journal of international medical research[Palmoplantar dermatoses: Diagnostic approach in primary care].
SemergenAcrokeratosis neoplastica (Bazex syndrome): Report of two cases and literature review.
Heliyon[Bazex syndrome: a rare paraneoplastic disease].
Medicina[Translated article] Complete Resolution of Bazex Syndrome After Surgical Treatment of Squamous Cell Carcinoma of the Larynx.
Actas dermo-sifiliograficasBazex Syndrome (Acrokeratosis Paraneoplastica): A Narrative Review of Pathogenesis, Clinical Manifestations, and Therapeutic Approaches.
CureusAnnular and acral/facial dyskeratotic paraneoplastic disorders.
Clinics in dermatologyBazex Syndrome in Acute Myeloid Leukemia.
Internal medicine (Tokyo, Japan)A case of Bazex syndrome with type 2 immune response.
European journal of dermatology : EJDAcrokeratosis Paraneoplastica (Bazex Syndrome): A Case Report.
Case reports in dermatologyBazex Syndrome Associated with Squamous Cell Carcinoma of the Lip: A Rare Paraneoplastic Acrokeratosis with Nail Dystrophy.
Skin appendage disordersAcrokeratosis of Bazex as a sign of thyroid cancer: first description and review of thyroid-associated paraneoplastic dermatoses.
European review for medical and pharmacological sciencesParaneoplastic Syndromes in Patients with Keratinocyte Skin Cancer.
CancersAn Unusual Encounter of Skin Condition Mimicking Acrokeratosis Paraneoplastica (Bazex Syndrome).
CureusA Case of Bazex Syndrome.
Internal medicine (Tokyo, Japan)A Review of Nail Changes in Acrokeratosis Paraneoplastica (Bazex Syndrome).
Skin appendage disorders[Hyperplasia-associated cutaneous paraneoplasia].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebieteAcrokeratosis paraneoplastica (Bazex syndrome) as the presenting sign of pancreatic adenocarcinoma.
BMJ case reportsA series of typical and atypical cases of Bazex syndrome: Identifying the red herring to avoid delaying cancer treatment.
Clinical case reportsAn Unconventional Presentation of Multiple Myeloma: Bazex Syndrome.
Turkish journal of haematology : official journal of Turkish Society of HaematologyFrom the Outside Looking in: Psoriasiform Dermatitis Presenting as a Paraneoplastic Syndrome for Pancreatic Adenocarcinoma.
Cureus[Detecting skin cancer: paraneoplastic skin diseases].
Der InternistParaneoplastic acrokeratosis (Bazex syndrome).
Medicina clinicaA case report of resolution of acrokeratosis paraneoplastica (Bazex syndrome) post resection of non-small-cell lung carcinoma.
SAGE open medical case reportsAcrokeratosis paraneoplastica (Bazex syndrome) with bullous lesions.
Indian journal of dermatology, venereology and leprologySynchronous Occurrence of Bazex Syndrome and Remitting Seronegative Symmetrical Synovitis with Pitting Edema Syndrome in a Patient with Lung Cancer.
Internal medicine (Tokyo, Japan)Acrokeratosis Can Be a Warning Sign of an Underlying Malignancy.
European journal of case reports in internal medicineAcrokeratosis paraneoplastica (Bazex syndrome) associated with peripheral T-cell lymphoma.
JAAD case reportsBazex syndrome in a patient with head and neck malignancy.
JAAPA : official journal of the American Academy of Physician AssistantsIs it just a psoriasiform dermatitis?
Dermatology online journalAcrokeratosis paraneoplastica (Bazex syndrome).
Ear, nose, & throat journalParaneoplastic acrokeratosis (Bazex syndrome): unusual association with in situ follicular lymphoma and response to acitretin.
Cutis[Hands and feet lesions].
La Revue de medecine interneAcrokeratosis paraneoplastica (Bazex syndrome) - a systematic review on risk factors, diagnosis, prognosis and management.
Journal of the European Academy of Dermatology and Venereology : JEADVAcrokeratosis Paraneoplastica Associated with Cervical Squamous Cell Carcinoma.
Case reports in dermatological medicineBazex Syndrome in Lung Squamous Cell Carcinoma: High Expression of Epidermal Growth Factor Receptor in Lesional Keratinocytes with Th2 Immune Shift.
Case reports in dermatologyBazex Syndrome with Hypoalbuminemia and Severe Ascites.
Case reports in oncologyAcrokeratosis paraneoplastica (Bazex syndrome) associated with metastatic cutaneous squamous cell carcinoma.
Internal medicine journalCase Report: Bazex Syndrome Associated With Pulmonary Adenocarcinoma.
MedicineAcitretin amelioration of Acrokeratosis Paraneoplastica (Bazex Syndrome) in cases of incurable squamous cell carcinoma of the hypopharynx.
Dermatology online journalPsoriasiform dermatitis and hepatocellular carcinoma: a new case of Bazex syndrome.
Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografiaAcute Onset of Acrokeratosis Paraneoplastica (Bazex Syndrome).
JAMA dermatologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Síndrome Bazex
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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.
- Acrokeratosis Paraneoplastica (Bazex Syndrome)-Like Dermatomyositis With Anti-PM/Scl Antibodies.
- The Importance and Challenges of Early Diagnosis of Paraneoplastic Skin Syndromes in Cancer Detection-A Review.
- A rare case of acrokeratosis paraneoplastica (Bazex syndrome) and a literature review.
- Recurrence of acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of metastatic recurrence of squamous cell carcinoma of the lung.
- [Palmoplantar dermatoses: Diagnostic approach in primary care].
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:166113(Orphanet)
- OMIM OMIM:301845(OMIM)
- MONDO:0010535(MONDO)
- GARD:838(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q4675787(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