Pular para o conteúdo
Doença de Paget do mamilo
ORPHA:180275CID-10 · C50.0CID-11 · 2E65.5DOENÇA RARA
Início adulta
Também conhecida comoDoença de Paget mamária

A doença de Paget do mamilo descreve uma apresentação rara de câncer de mama, observada mais frequentemente em mulheres com idade entre 50 e 60 anos, manifestando-se com drenagem e prurido no mamilo, eritema, mamilo crostoso e escoriado, placas espessadas e hiperpigmentação (com menor frequência). Ela se deve a células tumorais que invadem o complexo aréolo-mamilar e representa de 1 a 3% de todos os novos diagnósticos de câncer de mama.

Última verificação: Fonte: não verificado (1 pendência)Em construçãohistóricoSugerir correção
Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

Mantido pelo Disease Twin100% com fonte · atualizado 25/09/2026Revisão médica pendente
Informacoes curadas por IA — podem conter imprecisoes

Visão geral

A Doença de Paget do mamilo (código MONDO:0015873, CID-10 C50.0) é uma apresentação clínica rara de câncer de mama, com prevalência estimada entre 1 e 9 casos por 1.000.000 de indivíduos e início observado tipicamente na idade adulta.[1]

Causas genéticas

Atualmente, não constam informações sobre padrão de herança ou genes causais formalmente determinados nos registros oficiais de base de dados sobre a condição.[1][2]

Diagnóstico

Existem atualmente 2 registros de testes genéticos catalogados na literatura ou bases científicas de referência associados a essa investigação diagnóstica.[1][2][3]

Tratamento e manejo

No Sistema Único de Saúde (SUS), o nível de integração para a condição está classificado como cobertura parcial. No âmbito do PCDT vigente de Câncer de Mama, pactuado pela Portaria Conjunta SAES-SECTICS/MS nº 17 (25 de novembro de 2024), são dispensados os medicamentos e esquemas terapêuticos: trastuzumabe + paclitaxel, trastuzumabe + vinorelbina, trastuzumabe entansina, trastuzumabe + pertuzumabe + paclitaxel, trastuzumabe + pertuzumabe + docetaxel, vinorelbina, 5-fluorouracil, fulvestranto, ciclofosfamida + doxorrubicina + 5-fluorouracil, docetaxel + carboplatina + trastuzumabe, cisplatina, docetaxel + carboplatina, docetaxel + gencitabina, doxorrubicina lipossomal e cisplatina + gencitabina.[4][4][4][4][4][4][4][4][4][4][4][4][4][4][4]

Tratamentos citados na literatura

As seguintes substâncias foram identificadas por meio de mineração de literatura científica biomédica em associação à Doença de Paget do mamilo, não se tratando de recomendações clínicas de uso ou prescrições: Tamoxifen (4 publicações), Imiquimod (3 publicações), Steroids (3 publicações), calcipotriene (3 publicações), Melanins (2 publicações), Estradiol (1 publicação), Estrone (1 publicação), Fluorescein (1 publicação), Glycogen (1 publicação) e Histamine (1 publicação).[3]

Fontes numeradas em Referências.

Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.

💬
EM LINGUAGEM SIMPLES

A doença de Paget do mamilo é um tipo raro de câncer de mama que atinge a pele do mamilo e da aréola, sendo mais frequente em mulheres entre 50 e 60 anos. Ela ocorre quando células tumorais infiltram a região mamilar, provocando coceira, vermelhidão, descamação, crostas e pequenas secreções locais. O diagnóstico exige a confirmação por exames médicos para diferenciar a lesão de problemas comuns de pele. No Brasil, o SUS oferece acompanhamento e dispensa medicamentos para o tratamento do câncer de mama por meio de seu protocolo clínico oficial.

📋
Informacoes curadas por IA — podem conter imprecisoes

A doença de Paget do mamilo descreve uma apresentação rara de câncer de mama, observada mais frequentemente em mulheres com idade entre 50 e 60 anos, manifestando-se com drenagem e prurido no mamilo, eritema, mamilo crostoso e escoriado, placas espessadas e hiperpigmentação (com menor frequência). Ela se deve a células tumorais que invadem o complexo aréolo-mamilar e representa de 1 a 3% de todos os novos diagnósticos de câncer de mama.

Publicações científicas
264 artigos
Último publicado: 2026 Feb 9
Medicamentos
1 com registro
Alendronato

Tem tratamento?

✓ 1 medicamento específico desta doença (registro ANVISA, FDA ou SUS/CEAF)
Ver detalhes, fases e interações →
ALENDRONATO

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.51
Europe
Início
Adult
🏥
SUS: Cobertura parcialScore: 50%
1 medicamento CEAFCID-10: C50.0
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

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

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico264PubMed
Últimos 10 anos200publicações
Pico202524 papers
Linha do tempo
2026Hoje · 2026🧪 1997Primeiro ensaio clínico📈 2025Ano 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

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

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.
3Fase 31
2Fase 21
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença de Paget do mamilo

🗺️

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

Onde estão os ensaios

Nenhum país com ensaio aberto no momento.

Pesquisa e ensaios clínicos

2 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

TRPS1 Expression Across the Spectrum of Paget Disease and Morphologic Mimics.

Applied immunohistochemistry &amp; molecular morphology : AIMM2026 Feb 23

Paget disease (PD) is histologically characterized by malignant glandular epithelial cells singly or in clusters within the epidermis. PD most commonly involves the nipple, though it may also occur at extramammary sites. Immunohistochemistry is helpful in highlighting PD and its extent. Differentiating PD from histologic mimics with overlapping immunophenotypes can be challenging. TRPS1, a GATA family transcription factor, has emerged as a breast origin marker with greater sensitivity than GATA3. In this study, we comprehensively examined the immunohistochemical expression of TRPS1 in mammary PD (n=29) with different biomarker profiles, including ER-/HER2+ (n=12), ER+/HER2+ (n=7), ER+/HER2- (n=6), and ER-/HER2- (n=4) cases, as well as benign and malignant histologic mimics and extramammary PD (EMPD). TRPS1 demonstrated strong to moderate nuclear staining of PD tumor cells across all ER/HER2 subgroups. Toker cells/Toker cell hyperplasia (n=11) also exhibited strong TRPS1 immunoreactivity, while melanoma in situ (n=10) and cutaneous sebaceous carcinoma (n=9) were essentially negative. Squamous cell carcinoma in situ (n=13) showed predominantly weak staining for TRPS1. Two cases of presumed intraepidermal metastases in patients with breast cancer demonstrated strong TRPS1 expression. Notably, TRPS1 highlighted PD tumor cells from background keratinocytes more distinctly compared with GATA3. Among EMPD cases, perivulvar (n=10) and periscrotal (n=5) cases were consistently positive, whereas perianal lesions (n=10) showed no/weak expression. In summary, TRPS1 demonstrates universal expression in mammary PD of all ER/HER2 subgroups and in Toker cells, and corroborates recent reports regarding the utility of TRPS1 in site-of-origin studies.

#2

Not All That Produces Pigment Is Melanoma: Direct Epidermotropic Infiltration of Breast Carcinoma in a Woman With Melanin Incontinence Mimicking Pigmentary Disorders.

International journal of surgical pathology2026 Feb 18

BackgroundLocal spread linked to breast carcinoma demonstrates a diverse spectrum of clinical presentations, including zosteriform, telangiectatic, and erysipeloid lesions. Pigmented epidermotropic infiltration resulting from breast carcinoma is seldom observed, occasionally posing diagnostic challenges owing to its resemblance to various other pigmentary disorders, such as malignant melanoma and other melanocytic lesions.Patient PresentationA 47-year-old woman presented with complaints of a hyperpigmented, crusted lesion involving the nipple-areolar region of the left breast, which on histopathology revealed an invasive tumor involving the dermis, composed of discohesive cells arranged in single files and linear cords, extending directly into the overlying epidermis in a pagetoid pattern with extensive pigment incontinence. On employing a comprehensive panel of special stains and immunohistochemical markers, the presence of classic Paget cells was ruled out, and a final diagnosis of direct epidermotropic infiltration by underlying invasive lobular carcinoma (ILC) of the breast was found.ConclusionEpidermotropic spread of ILC with associated pigment incontinence is a rare histopathological pattern that can closely mimic both pigmented mammary Paget disease and melanocytic disorders. The distinction is critical, as these entities differ significantly in their pathogenesis, prognosis, and management.

#3

Diagnosis and management of nipple-areolar complex conditions in postmenopausal women: a retrospective study highlighting multidisciplinary care needs.

International journal of women's dermatology2026 Mar

Nipple conditions in postmenopausal women are numerous and challenging to diagnose, requiring multidisciplinary collaboration. There are no large cohort studies detailing the epidemiology, symptoms, diagnoses, and disease trajectories for postmenopausal women with nipple complaints. This single-institution retrospective study aimed to describe postmenopausal nipple complaints, associated symptoms, diagnoses, and involved medical care teams. Medical charts of female patients over 49 years old with nipple-related diagnoses were reviewed. Data on demographics, symptoms, medical specialties, diagnostics, final diagnoses, and treatments were collected. Physician-patient encounters (N = 296) for 174 unique patients ages 50 to 92 (average 70) were analyzed. Nipple inversion, pain, and dermatitis were the most common diagnoses. Primary care physicians (PCPs) cared for the majority of patients (73% first seen by PCP), with dermatologists, surgeons, and breast health specialists involved in over 25% of encounters. PCPs often referred patients to specialists when patients presented with a nipple rash with ulceration or an inverted nipple with discharge. Most patients (63%) received reassurance about benign or self-limited conditions, while others were treated with oral antibiotics, topical medications, or surgical management depending on the diagnosis. This is a single institution retrospective chart review. Most postmenopausal women with nipple complaints are diagnosed with inversion, pain, or dermatitis. This retrospective chart review highlights the need for multidisciplinary care of these patients, with the primary care provider serving as the frontline care provider for women with skin conditions of the nipple-areolar complex.

#4

Unilateral Giant Seborrheic Keratosis of the Nipple-Areola Complex in a Young Woman: A Case Report.

Cureus2026 Jan

Seborrheic keratosis is a common benign epidermal tumor that affects individuals of various ages and may arise on both sun-exposed and non-sun-exposed areas. Involvement of the nipple-areola complex is rare and may pose a diagnostic challenge due to its clinical resemblance to malignant or premalignant breast conditions, particularly Paget disease of the breast. We report the case of a 36-year-old woman who presented with a long-standing, progressively enlarging unilateral pigmented verrucous lesion involving the left nipple-areola complex, with onset and marked growth during two consecutive pregnancies. Clinical examination, dermoscopic evaluation, and breast imaging were performed, followed by histopathological analysis, which confirmed the diagnosis of acanthotic, pigmented seborrheic keratosis without evidence of malignancy. Given the benign nature of the lesion and patient preference, conservative management was selected with close dermatologic follow-up. This case highlights the importance of considering seborrheic keratosis in the differential diagnosis of nipple-areola lesions, even in young patients, and underscores the essential role of histopathological examination in establishing an accurate diagnosis and guiding individualized management.

#5

An Integrative review of imaging approaches and clinical management in Paget's disease of the breast.

Current problems in diagnostic radiology2026 Feb 03

Paget disease of the breast represents a rare variant of breast cancer, primarily affecting the epidermis of the nipple-areola complex. It typically manifests as unilateral eczema or ulceration of the nipple. In over 90% of instances, it is concomitant with an underlying breast malignancy, such as ductal carcinoma in situ, invasive carcinoma, or both. The diagnosis is predominantly clinical, and is corroborated through histopathological examination. Imaging is pivotal in identifying any associated malignancy. Although mammography may yield normal results in up to 50% of cases, breast MRI provides superior sensitivity, particularly in patients lacking palpable abnormalities, by delineating the full extent of the disease. This review examines the etio-pathogenesis, clinical manifestations, and imaging characteristics across ultrasound, mammography, and MRI, and proposes an algorithmic imaging approach for the diagnosis and subsequent management of Paget disease of the breast.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC19 artigos no totalmostrando 200

2026

TRPS1 Expression Across the Spectrum of Paget Disease and Morphologic Mimics.

Applied immunohistochemistry &amp; molecular morphology : AIMM
2026

Not All That Produces Pigment Is Melanoma: Direct Epidermotropic Infiltration of Breast Carcinoma in a Woman With Melanin Incontinence Mimicking Pigmentary Disorders.

International journal of surgical pathology
2026

Diagnosis and management of nipple-areolar complex conditions in postmenopausal women: a retrospective study highlighting multidisciplinary care needs.

International journal of women's dermatology
2026

Unilateral Giant Seborrheic Keratosis of the Nipple-Areola Complex in a Young Woman: A Case Report.

Cureus
2026

An Integrative review of imaging approaches and clinical management in Paget's disease of the breast.

Current problems in diagnostic radiology
2025

Case Report: Mammary Paget's disease with multifocal microinvasive carcinoma and extensive lymph node metastasis: therapeutic challenges and insights from a case of stage pT1mic pN3c cM0.

Frontiers in oncology
2025

[A Case of Paget's Disease of the Breast in a Young Adult Woman].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2025

Unilateral Nevoid Hyperkeratosis of the Nipple and Areola: A Case Report of a Rare Entity.

Cureus
2025

Multimodal Ultrasound Manifestations of a Central Intraductal Papilloma at the Nipple and Its Causes of Misdiagnosis: A Case Report.

Cureus
2025

Unilateral, Episodic, Transient Blanching of Nipple With Pain in a Male: A Case Report.

WMJ : official publication of the State Medical Society of Wisconsin
2025

Clinical and Pathological Characteristics of the Mammary Paget's Disease: A Single-Center Retrospective Study in Japan.

Cancer investigation
2025

Dermoscopy of Mammary Paget's Disease: A Case Report and Literature Review.

Cureus
2025

Possible role of anastrozole-induced hormonal alterations in pathogenesis of mammary apocrine carcinoma and follicular lymphoma: a case report and review of the literature.

Journal of medical case reports
2025

Squamous Cell Carcinoma In Situ (SCCIS) of the Nipple Following Breast Conserving Therapy: Case Report.

Hawai'i journal of health &amp; social welfare
2025

Breast and Nipple Dermatoses During Lactation.

The Australasian journal of dermatology
2025

Dermatologic Mimickers of Paget's Disease of the Breast: A Systematic Review.

Journal of cutaneous medicine and surgery
2025

Multimodal Imaging Detection of Difficult Mammary Paget Disease: Dermoscopy, Reflectance Confocal Microscopy, and Line-Field Confocal-Optical Coherence Tomography.

Diagnostics (Basel, Switzerland)
2025

Pigmented Paget's Disease of Nipple Mimicking Malignant Melanoma.

Indian dermatology online journal
2025

Pagetoid squamous cell carcinoma in situ (Pagetoid bowen's disease) of the nipple in an older male patient: a case report and literature review.

BMC geriatrics
2025

Mammary Paget's Disease of the Nipple: A Case Report.

Case reports in oncology
2025

Unilateral breast Darier disease: A case report and literature review.

Medicine international
2025

Mammary Paget's disease in a young woman: A rare occurrence.

Radiology case reports
2025

Dermatologic diseases of the breast, areola and nipple.

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

A Rare Case Of Pigmented Mammary Paget Disease.

Clinical, cosmetic and investigational dermatology
2025

Insights Into Mammary and Extramammary Paget's Disease: Diagnosis, Management, and Recent Advances.

Cureus
2025

Utility of contrast-enhanced magnetic resonance imaging for planning of surgical procedure in Paget's disease of the breast.

Surgery today
2025

Diagnostic imaging challenges of mammary Paget's disease presenting with subtle clinical and imaging features: A case report.

Radiology case reports
2024

Case report: Illustrating associated malignancies in Paget's disease using contrast-enhanced mammography.

Frontiers in oncology
2024

Reflectance Confocal Microscopy Can Help in Detecting Clinically Silent Mammary Paget's Disease.

Diagnostics (Basel, Switzerland)
2025

Invasive Squamous Cell Carcinoma of the Nipple: Case Report With Literature Review.

The American Journal of dermatopathology
2024

Primary nipple melanoma in a patient with breast cancer: A diagnosis to consider.

Radiology case reports
2024

Simultaneous Occurrence of Two Ectopic Extramammary Paget Disease Lesions: A Case Report.

Cureus
2024

Mammary Paget's Disease Mimicking Benign and Malignant Dermatological Conditions: Clinical Challenges and Diagnostic Considerations.

Cureus
2025

Nipple adenoma: A clinicopathological study over a period of six years in tertiary care center.

Indian journal of pathology &amp; microbiology
2024

Adenoma of the Nipple: A Case Report.

Cureus
2024

Mammary Paget's Disease as a Sign of Local Recurrence Two Decades Following Breast Conservation and Adjuvant Therapy for Early Stage Breast Cancer.

Cureus
2024

Primary Invasive Paget's Disease in a Clinical Practice: A Case Report.

Cureus
2024

Invasive cribriform carcinoma of the breast presenting as an erythematous papule on the nipple: A case report.

Clinical case reports
2024

Case report: Metastatic ovarian mucinous carcinoma to the breast: diagnostic challenges and pitfalls.

Frontiers in oncology
2024

A case of giant nipple adenoma.

Surgical case reports
2024

Paget's Presentation of High-Grade Ductal Carcinoma In Situ (DCIS) in a Very Young Female With Breast Cancer 2 (BRCA2) Mutation.

Cureus
2024

Unusual presentation of intraductal papilloma on the nipple: A case report.

International journal of surgery case reports
2024

Multimodality Imaging of Benign and Malignant Diseases of the Nipple-Areolar Complex.

Radiographics : a review publication of the Radiological Society of North America, Inc
2024

'Eczematous' dermatitis of the nipple: clinical and histopathological differential diagnosis of Paget disease.

Pathology
2024

Imaging Patterns in Breast Cancer for Women Under 40 Years: A Descriptive Cohort Study.

Journal of epidemiology and global health
2024

A painful erythematous plaque on the abdomen of a woman with ipsilateral breast cancer.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Primary melanoma of the nipple: Report of 10 cases including coexistence with Paget's disease.

Pathology, research and practice
2024

Dense Lichenoid Inflammation in Paget Disease: A Diagnostic Pitfall.

The American Journal of dermatopathology
2024

Melanocyte colonization and pigmentation of breast carcinoma: A case report.

Journal of cutaneous pathology
2024

Paget's disease of the nipple with underlying occult invasive carcinoma detected by magnetic resonance imaging and second-look ultrasound: Case report.

International journal of surgery case reports
2023

Clinicopathological profile of breast cancer from Chhattisgarh India: A single-center hospital-based study.

Journal of family medicine and primary care
2024

Melanoma Mimicker: Pigmented Mammary Paget Disease in a Man.

The American Journal of dermatopathology
2023

A Pictorial Exploration of Mammary Paget Disease: Insights and Perspectives.

Cancers
2023

Nipple Lesions of the Breast: An Update on Morphologic Features, Immunohistochemical Findings and Differential Diagnosis.

Advances in anatomic pathology
2023

Extensive Paget's disease of the nipple: Case report.

International journal of surgery case reports
2023

Studying the Presentation of Paget Disease of the Breast.

Cureus
2023

Nipple Adenoma: Clinicopathologic Characterization of 50 Cases.

The American journal of surgical pathology
2023

Oncoplastic surgery for Paget's disease of the breast.

Frontiers in oncology
2023

Rare Paget's Disease Associated With Breast Cancer in a 70-Year-Old Male.

Cureus
2023

Extra-mammary Paget's disease of the penis.

International journal of STD &amp; AIDS
2023

Skin diseases of the nipple and areola complex: A case series study from China.

Frontiers in medicine
2023

[Pathoanatomical algorithm for differential diagnosis of Paget's disease of the breast].

Arkhiv patologii
2023

Paget disease of the breast with underlying invasive ductal cancer in neurofibromatosis type 1: a case report.

Translational cancer research
2023

[Paget’s disease of the nipple].

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
2022

Mammary Paget's Disease Presenting as an Annular Plaque.

Acta dermatovenerologica Croatica : ADC
2023

Toker Cell Hyperplasia in the Nipple-Areolar Complex of Transmasculine Individuals.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2023

Basosquamous Cell Carcinoma of the Nipple-Areola Complex-Report of a Case.

Medicina (Kaunas, Lithuania)
2023

Invasive breast cancer with Paget's disease: A rare case report from a tertiary facility in Northern Tanzania.

SAGE open medical case reports
2023

Paget's disease of the breast: diagnosis and management.

British journal of hospital medicine (London, England : 2005)
2023

Nipple eczema: A systematic review and practical recommendations.

Journal of the European Academy of Dermatology and Venereology : JEADV
2023

Nipple-Sparing Mastectomy After Neoadjuvant Chemotherapy: Definitive Results with a Long-Term Follow-Up Evaluation.

Annals of surgical oncology
2022

Treatment of Mammary Paget Disease: A systematic review and meta-analysis of real-world data.

International journal of surgery (London, England)
2022

Large Mammary Paget Disease without Underlying Breast Carcinoma.

Plastic and reconstructive surgery. Global open
2022

A monolateral pigmented lesion of the nipple.

Clinical and experimental dermatology
2022

[Management of nipple-areolar complex anomalies].

Revue medicale de Liege
2022

Multidisciplinary management of mammary Paget's disease: recommendations to optimize oncological and aesthetic outcomes.

Annali italiani di chirurgia
2023

The nipple-areolar complex: anatomy, methods and pathologic findings, between senologist and dermatologist.

Journal of ultrasound
2022

Local recurrence of mammary Paget's disease after nipple-sparing mastectomy and implant breast reconstruction: a case report and literature review.

World journal of surgical oncology
2023

Nipple Adenoma: A Benign Disease With a Suspicious Presentation.

The American surgeon
2022

Imprint Cytology Can Be a Better Option for Diagnosis of Mammary Paget's Disease!!!: A Case Report with Review.

Journal of cutaneous and aesthetic surgery
2022

[Therapeutic peculiarities in diseases of the nipple skin].

Dermatologie (Heidelberg, Germany)
2022

Nipple Adenoma: Correlation of Imaging Findings and Histopathology.

Journal of breast imaging
2022

[Surgical management of nipple areola complex in central breast cancer].

Zhonghua zhong liu za zhi [Chinese journal of oncology]
2022

Calciphylaxis and Ulcerative Lesions of Bilateral Nipple-Areolar Complex Due to Alcoholic Liver Disease.

The Indian journal of radiology &amp; imaging
2022

Mammary Paget's Disease: An Update.

Cancers
2022

A peculiar case of Paget's disease of the breast.

Annali italiani di chirurgia
2022

A tale of three common nipple diseases.

Proceedings (Baylor University. Medical Center)
2022

Nipple Adenoma: Case Report of a Rare Entity.

Cureus
2022

A rare breast cancer in a patient with pierced nipples.

JAAPA : official journal of the American Academy of Physician Assistants
2022

Pathological and imaging features of Paget's disease and nipple adenoma: a comparative study.

Gland surgery
2023

Clinically guided core biopsy and cutaneous punch biopsy in the evaluation of breast lesions: a necessary test or an obsolete skill?

Irish journal of medical science
2022

Keratin 7 and ERBB2 Negative Mammary Paget Disease Without an Underlying in Situ and Invasive Carcinoma.

International journal of surgical pathology
2021

Pigmentary mammary Paget disease: Clinical, dermoscopical and histological challenge.

Dermatology reports
2021

The Great Imitator: Atypical Cutaneous Manifestations of Primary Syphilitic Chancre.

Chinese medical sciences journal = Chung-kuo i hsueh k'o hsueh tsa chih
2021

Invasive Paget's Disease of the Breast: Rash or Recurrence?

Cureus
2021

Paget's disease of the breast in male with underlying invasive ductal carcinoma: A case report with review of literature.

Annals of medicine and surgery (2012)
2021

Modified Grisotti flap technique in centrally located breast cancer: case report.

Gland surgery
2022

Paget's disease of nipple with dermal invasion: A case report.

Cancer reports (Hoboken, N.J.)
2021

Surgical management of erosive adenomatosis of the nipple: A case report.

International journal of surgery case reports
2021

Comparison of the Magnetic Resonance Imaging Findings of Paget's Disease of the Breast and Malignant Tumor Invasion of the Nipple-Areola Complex.

European journal of breast health
2021

Pagets disease of the breast - observational retrospective study of the past 20 years.

Rozhledy v chirurgii : mesicnik Ceskoslovenske chirurgicke spolecnosti
2021

Differentiating pagetoid Bowen disease from Paget disease on the nipple-areola complex: Two unique, challenging cases.

Journal of cutaneous pathology
2022

Delayed presentation of inflammatory breast carcinoma during the COVID-19 pandemic.

European surgery : ACA : Acta chirurgica Austriaca
2021

Adenoma of the nipple: a mimic of breast malignancy.

Dermatology online journal
2021

A new technique for the histological diagnosis of Paget's disease of the breast using a semiautomated core needle biopsy with a 14-gauge needle.

La Radiologia medica
2021

Not All Cases of Mammary Paget's Disease are Cytokeratin-7 Positive: A Challenging Diagnosis!

International journal of surgical pathology
2021

Magnetic resonance imaging in the evaluation of pathologic nipple discharge: indications and imaging findings.

The British journal of radiology
2020

[A Case of Male Mammary' Paget's Disease].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2020

Male Mammary Paget Disease: A Tale of 2 Contrasting Cases.

The American Journal of dermatopathology
2021

[Persistent, unilateral eczema of the nipple in a man].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
2021

Toker cell hyperplasia in Zuska disease: A tricky association.

Journal of cutaneous pathology
2022

Cutaneous Squamous Cell Carcinoma of the Nipple Presenting as Paget's Disease.

The American surgeon
2020

Invasive Cancer Confined to the Nipple of the Conserved Breast: A Case Report.

Case reports in oncology
2021

The nipple-areolar complex: comprehensive imaging review.

Clinical radiology
2021

Are pathogenic BRCA1 mutations associated with parotid mucoepidermoid carcinoma? A case report.

Oral surgery, oral medicine, oral pathology and oral radiology
2020

Erosive Adenomatosis of the Nipple: A Clinical Diagnostic Challenge.

Clinical, cosmetic and investigational dermatology
2020

Case for diagnosis. Phagedenic ulcer on the thorax.

Anais brasileiros de dermatologia
2020

Paget Disease of the Breast in Pregnancy and Lactation.

Advances in experimental medicine and biology
2020

Establishment and characterization of breast cancer organoids from a patient with mammary Paget's disease.

Cancer cell international
2020

Advanced lesions of synchronous bilateral mammary Paget's disease: a case report.

Journal of medical case reports
2020

Nipple Ultrasound: A Pictorial Essay.

Korean journal of radiology
2020

Invasive Breast Carcinoma Arising in a Nipple Adenoma After 15 Years: Report of a Rare Case and Literature Review.

Cureus
2020

Corrigendum: Unusual case of Paget disease of the nipple diagnosed by nipple scraping cytology.

Dermatology online journal
2020

Unusual case of Paget disease of the nipple diagnosed by nipple scraping cytology.

Dermatology online journal
2020

Angiogenesis in mammary Paget disease: histopathological analyses of blood vessel density and angiogenic factors.

Diagnostic pathology
2020

[Pathology of the nipple-areola complex : I. Paget's disease of the nipple, variants, and differential diagnoses].

Der Pathologe
2020

Recurrence of Breast Carcinoma as Paget's Disease of the Skin along the Core Needle Biopsy Tract after Skin-Sparing Mastectomy.

Journal of breast cancer
2020

Herpes Simplex Mastitis in an Adolescent Woman: Clinical and Ultrasound Features.

Taehan Yongsang Uihakhoe chi
2020

Clinical and sonographic features of nipple lesions.

Medicine
2020

Practical approach to extensive cutaneous spread without any underlying malignancy: a rare presentation of Paget's disease of breast.

BMJ case reports
2020

A rare case of Bowen's disease of the nipple: Literature review and management pathway.

The breast journal
2020

Pure Ductal Carcinoma in Situ in The Male Breast: A Rare Entity.

European journal of breast health
2020

Paget's disease of the nipple in a Her2-positive breast cancer xenograft model.

Breast cancer research and treatment
2019

Paget’s disease of the breast without associated ductal carcinoma: case report and review of the literature.

Revista colombiana de obstetricia y ginecologia
2019

Florid papillomatosis of the nipple.

BMJ case reports
2020

Mammary Paget's Disease of the Male Breast: A Rare Case With an Unusual Immunohistochemical Profile.

International journal of surgical pathology
2019

Unilateral nipple hyperkeratosis.

JAAD case reports
2019

Surgical management of ductal carcinoma in situ of the breast: A large retrospective study from a single institution.

The breast journal
2019

Dermoscopic features of mammary Paget's disease: a retrospective case-control study by the International Dermoscopy Society.

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

Isolated invasive ductal carcinoma of the nipple-areolar complex: A rare occurrence yet to be reported in current literature.

The breast journal
2019

Nipple and areola lesions: review of dermoscopy and reflectance confocal microscopy features.

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

Extramammary Paget disease.

Dermatology online journal
2019

[The Diagnostic Problem of the Automated Breast Volume Scanner(ABUS) for Paget Disease-A Case Report].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2019

Extramammary Paget Disease-A Challenging Case.

The American Journal of dermatopathology
2019

Online Information-Seeking About Potential Breast Cancer Symptoms: Capturing Online Behavior With an Internet Browsing Tracking Tool.

Journal of medical Internet research
2019

Incidental discovery of follicular lymphoma by sentinel lymph node biopsy and skin-sparing mastectomy for Paget's disease associated with invasive breast cancer.

Journal of surgical case reports
2018

[A Case of Invasive Ductal Carcinoma with Paget's Disease of the Breast].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2018

Prognostic significance of mammary Paget's disease in Chinese women: a 10-year, population-based, matched cohort study.

OncoTargets and therapy
2019

Mammary Paget's Disease of the Nipple: Relatively Common but Still Unknown to Many.

Korean journal of family medicine
2019

The utility of nipple scraping in the diagnosis of Paget disease of the breast.

Diagnostic cytopathology
2019

Nipple and areola lesions: Dermoscopy and reflectance confocal microscopy features.

Journal of the American Academy of Dermatology
2019

Skin diseases of the breast and nipple: Benign and malignant tumors.

Journal of the American Academy of Dermatology
2018

Squamous Cell Carcinoma Arising within Verruca Vulgaris on the Nipple.

Case reports in dermatological medicine
2018

Bilateral mammary Paget disease in a young adult female.

Radiology case reports
2018

Mammary Paget's disease without underlying malignancy of the breast.

Yeungnam University journal of medicine
2019

Unilateral nipple erosion with acantholysis.

International journal of dermatology
2018

Pigmented mammary Paget disease mimicking cutaneous melanoma.

International journal of dermatology
2018

Sonographic visualization of nipple blood flow can help differentiate Paget disease from benign eczematous nipple lesions.

PloS one
2018

Practical consensus recommendatons for Paget's disease in breast cancer.

South Asian journal of cancer
2018

Paget's disease of the male breast: case report and a point of view from actual literature.

Il Giornale di chirurgia
2018

[Paget's disease of ectopic breast].

Annales de dermatologie et de venereologie
2018

Invasive Solid Papillary Carcinoma of the Nipple With Pagetoid Extension and Nodal Metastasis.

International journal of surgical pathology
2018

Pigmented Paget's disease of the nipple mistaken for melanoma in situ: a diagnostic pitfall for the unwary.

Pathology
2018

Nipple adenoma in a 2-year-old boy.

Pediatric dermatology
2018

Nipple-Invasive Primary Carcinomas: Clinical, Imaging, and Pathologic Features of Breast Carcinomas Originating in the Nipple.

Archives of pathology &amp; laboratory medicine
2017

[A Case of Paget-Type Recurrence 20 Years after Breast Conserving Surgery for Invasive Ductal Carcinoma].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2017

[A Case of Adenoma of the Nipple Suspected of Breast Cancer].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2017

Paget's Disease of the Breast: A dangerous imitator of eczema.

Sultan Qaboos University medical journal
2018

Invasive papillary breast cancer with Paget's disease: first reported association and literature review.

BMJ case reports
2018

Solid papillary carcinoma of the breast with an associated Cytokeratin 7- negative Paget's disease of the nipple. Report of a first case.

The breast journal
2017

Reflectance confocal microscopy of mammary Paget disease.

Dermatology practical &amp; conceptual
2017

Erythematous, friable nipple with loss of protrusion · history of breastfeeding · Dx?

The Journal of family practice
2018

Invasion in breast lesions: the role of the epithelial-stroma barrier.

Histopathology
2018

Nipple-sparing mastectomy as treatment for patients with ductal carcinoma in situ: A 10-year follow-up study.

The breast journal
2017

Paget's Breast Disease: A Case Report and Review of the Literature.

Frontiers in surgery
2017

MOHS micrographic surgery for treating erosive adenoma of the nipple: a case report and review of the literature.

International journal of dermatology
2017

Pyoderma Gangrenosum after Breast Mastectomy and Primary Rectus Abdominis Flap Reconstruction.

The Tokai journal of experimental and clinical medicine
2017

[Erosive lesion of the nipple].

The Pan African medical journal
2017

Invasive Paget's disease of the male nipple: a case report.

Journal of surgical case reports
2017

Clinical and Sonographic Features of Mammary Paget's Disease.

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2017

An atypical presentation of Paget's Disease of the breast without nipple involvement: Case report and review of the literature.

Pathology, research and practice
2017

Dermoscopy as a Supportive Instrument in the Early Recognition of Erosive Adenomatosis of the Nipple and Mammary Paget's Disease.

Annals of dermatology
2017

[Hyperkeratotic lesion of the nipple and areola].

Presse medicale (Paris, France : 1983)
2017

A common cancer in an uncommon location: A case report of squamous cell carcinoma of the nipple.

International journal of surgery case reports
2017

Paget disease of the male breast.

Dermatology online journal
2017

[Paget's disease of the nipple].

The Pan African medical journal
2017

Occurence of Paget's Disease in a Reconstructed Nipple 25 years after Performing Nipple Sharing Technique.

Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der V...
2016

Glandular Paget's Disease of the Male Nipple.

Annals of dermatology
2016

Paget's disease of the male breast in the 21st century: A systematic review.

Breast (Edinburgh, Scotland)
2016

An unusual case of mammary Paget’s disease in a woman with psoriasis.

Journal of biological regulators and homeostatic agents
2016

Nipple adenoma in a female patient presenting with persistent erythema of the right nipple skin: case report, review of the literature, clinical implications, and relevancy to health care providers who evaluate and treat patients with dermatologic conditions of the breast skin.

BMC dermatology
2016

Paget's Disease of Nipple in Male Breast with Cancer.

Journal of clinical and diagnostic research : JCDR
2015

Challenges in Diagnosis and Management of Paget's Disease of the Breast-a Retrospective Study.

The Indian journal of surgery
2016

Cytokeratin 7-negative mammary Paget's disease: A diagnostic pitfall.

Pathology, research and practice
2015

Paget's disease of the male breast: a case report.

Surgical case reports
2016

Mimics of Mammary Paget Disease: A Rogues' Gallery.

International journal of surgical pathology
2015

Diagnosis and treatment of Paget's disease of the breast: an analysis of 72 cases.

International journal of clinical and experimental medicine
2015

Synchronous bilateral primary breast malignant phyllodes tumor and carcinoma of the breast with Paget's disease: a case report and review of the literature.

International journal of clinical and experimental medicine
2016

Pigmented lesion of the nipple - a clinicopathological challenge.

Dermatology online journal
2015

[Rare benign breast tumors including Abrikossoff tumor (granular cell tumor), erosive adenomatosis of the nipple, cytosteatonecrosis, fibromatosis (desmoid tumor), galactocele, hamartoma, hemangioma, lipoma, juvenile papillomatosis, pseudoangiomatous hyperplasia, and syringomatous adenoma: Guidelines for clinical practice].

Journal de gynecologie, obstetrique et biologie de la reproduction

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Doença de Paget do mamilo.

É de uma associação que acompanha esta doença? Fale com a gente →

Vai consultar um especialista? Confirme o registro dele no conselho.
Conselhos e sociedades

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

Protocolo oficial relacionado (Ministério da Saúde)PCDT: Doença de Paget

Existe um documento oficial para "Doença de Paget". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.

Respostas geradas por IA a partir das fontes citadas

É uma apresentação rara de câncer de mama que se desenvolve pela infiltração de células tumorais na aréola e no mamilo. Ela representa cerca de 1% a 3% dos novos diagnósticos de câncer de mama e afeta predominantemente adultos entre 50 e 60 anos.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

5 fontes citadas no texto · 8 publicações do grafo RarasNet (PubMed) · 5 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. MONDO
    purl.obolibrary.org
  2. TRPS1 Expression Across the Spectrum of Paget Disease and Morphologic Mimics.
    Applied immunohistochemistry &amp; molecular morphology : AIMM2026PMID 41725031
  3. GARD:7303
    GARD (NIH)
  4. Q1572384
    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.

Citar este verbete

Raras. (2026). Doença de Paget do mamilo. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/doenca-de-paget-do-mamilo

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Doença de Paget do mamilo
Compêndio · Raras BR

Doença de Paget do mamilo

ORPHA:180275 · MONDO:0015873
🇧🇷 Brasil SUS
CEAF
1BAlendronato
Geral
Prevalência
1-9 / 1 000 000
CID-10
C50.0 · Neoplasia maligna do mamilo e aréola
CID-11
Início
Adult
Prevalência
0.51 (Europe)
MedGen
UMLS
C0030185
EuropePMC
Wikidata
Wikipedia

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Rara