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.
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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
Diagnóstico
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]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
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.
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.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
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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 — Doença de Paget do mamilo
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Publicações mais relevantes
TRPS1 Expression Across the Spectrum of Paget Disease and Morphologic Mimics.
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.
Not All That Produces Pigment Is Melanoma: Direct Epidermotropic Infiltration of Breast Carcinoma in a Woman With Melanin Incontinence Mimicking Pigmentary Disorders.
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.
Diagnosis and management of nipple-areolar complex conditions in postmenopausal women: a retrospective study highlighting multidisciplinary care needs.
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.
Unilateral Giant Seborrheic Keratosis of the Nipple-Areola Complex in a Young Woman: A Case Report.
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.
An Integrative review of imaging approaches and clinical management in Paget's disease of the breast.
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
Management of Extramammary Paget Disease of the Male Genital Region: A Narrative Review and Two Case Reports.
Unilateral Giant Seborrheic Keratosis of the Nipple-Areola Complex in a Young Woman: A Case Report.
An Integrative review of imaging approaches and clinical management in Paget's disease of the breast.
Practical Interpretation of Bone Scintigraphy: Metastases, Fractures, and Beyond.
Clinicopathologic features and prognostic factors of surgically treated extramammary Paget disease of the vulva: A retrospective cohort analysis.
📚 EuropePMC19 artigos no totalmostrando 200
TRPS1 Expression Across the Spectrum of Paget Disease and Morphologic Mimics.
Applied immunohistochemistry & molecular morphology : AIMMNot 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 pathologyDiagnosis and management of nipple-areolar complex conditions in postmenopausal women: a retrospective study highlighting multidisciplinary care needs.
International journal of women's dermatologyUnilateral Giant Seborrheic Keratosis of the Nipple-Areola Complex in a Young Woman: A Case Report.
CureusAn Integrative review of imaging approaches and clinical management in Paget's disease of the breast.
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Frontiers in oncology[A Case of Paget's Disease of the Breast in a Young Adult Woman].
Gan to kagaku ryoho. Cancer & chemotherapyUnilateral Nevoid Hyperkeratosis of the Nipple and Areola: A Case Report of a Rare Entity.
CureusMultimodal Ultrasound Manifestations of a Central Intraductal Papilloma at the Nipple and Its Causes of Misdiagnosis: A Case Report.
CureusUnilateral, Episodic, Transient Blanching of Nipple With Pain in a Male: A Case Report.
WMJ : official publication of the State Medical Society of WisconsinClinical and Pathological Characteristics of the Mammary Paget's Disease: A Single-Center Retrospective Study in Japan.
Cancer investigationDermoscopy of Mammary Paget's Disease: A Case Report and Literature Review.
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Journal of medical case reportsSquamous Cell Carcinoma In Situ (SCCIS) of the Nipple Following Breast Conserving Therapy: Case Report.
Hawai'i journal of health & social welfareBreast and Nipple Dermatoses During Lactation.
The Australasian journal of dermatologyDermatologic Mimickers of Paget's Disease of the Breast: A Systematic Review.
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Journal of epidemiology and global healthA painful erythematous plaque on the abdomen of a woman with ipsilateral breast cancer.
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Irish journal of medical scienceKeratin 7 and ERBB2 Negative Mammary Paget Disease Without an Underlying in Situ and Invasive Carcinoma.
International journal of surgical pathologyPigmentary mammary Paget disease: Clinical, dermoscopical and histological challenge.
Dermatology reportsThe Great Imitator: Atypical Cutaneous Manifestations of Primary Syphilitic Chancre.
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CureusPaget's disease of the breast in male with underlying invasive ductal carcinoma: A case report with review of literature.
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International journal of surgery case reportsComparison 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 healthPagets disease of the breast - observational retrospective study of the past 20 years.
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European surgery : ACA : Acta chirurgica AustriacaAdenoma of the nipple: a mimic of breast malignancy.
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International journal of surgical pathologyMagnetic resonance imaging in the evaluation of pathologic nipple discharge: indications and imaging findings.
The British journal of radiology[A Case of Male Mammary' Paget's Disease].
Gan to kagaku ryoho. Cancer & chemotherapyMale Mammary Paget Disease: A Tale of 2 Contrasting Cases.
The American Journal of dermatopathology[Persistent, unilateral eczema of the nipple in a man].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebieteToker cell hyperplasia in Zuska disease: A tricky association.
Journal of cutaneous pathologyCutaneous Squamous Cell Carcinoma of the Nipple Presenting as Paget's Disease.
The American surgeonInvasive Cancer Confined to the Nipple of the Conserved Breast: A Case Report.
Case reports in oncologyThe nipple-areolar complex: comprehensive imaging review.
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Oral surgery, oral medicine, oral pathology and oral radiologyErosive Adenomatosis of the Nipple: A Clinical Diagnostic Challenge.
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Anais brasileiros de dermatologiaPaget Disease of the Breast in Pregnancy and Lactation.
Advances in experimental medicine and biologyEstablishment and characterization of breast cancer organoids from a patient with mammary Paget's disease.
Cancer cell internationalAdvanced lesions of synchronous bilateral mammary Paget's disease: a case report.
Journal of medical case reportsNipple Ultrasound: A Pictorial Essay.
Korean journal of radiologyInvasive Breast Carcinoma Arising in a Nipple Adenoma After 15 Years: Report of a Rare Case and Literature Review.
CureusCorrigendum: Unusual case of Paget disease of the nipple diagnosed by nipple scraping cytology.
Dermatology online journalUnusual case of Paget disease of the nipple diagnosed by nipple scraping cytology.
Dermatology online journalAngiogenesis in mammary Paget disease: histopathological analyses of blood vessel density and angiogenic factors.
Diagnostic pathology[Pathology of the nipple-areola complex : I. Paget's disease of the nipple, variants, and differential diagnoses].
Der PathologeRecurrence of Breast Carcinoma as Paget's Disease of the Skin along the Core Needle Biopsy Tract after Skin-Sparing Mastectomy.
Journal of breast cancerHerpes Simplex Mastitis in an Adolescent Woman: Clinical and Ultrasound Features.
Taehan Yongsang Uihakhoe chiClinical and sonographic features of nipple lesions.
MedicinePractical approach to extensive cutaneous spread without any underlying malignancy: a rare presentation of Paget's disease of breast.
BMJ case reportsA rare case of Bowen's disease of the nipple: Literature review and management pathway.
The breast journalPure Ductal Carcinoma in Situ in The Male Breast: A Rare Entity.
European journal of breast healthPaget's disease of the nipple in a Her2-positive breast cancer xenograft model.
Breast cancer research and treatmentPaget’s disease of the breast without associated ductal carcinoma: case report and review of the literature.
Revista colombiana de obstetricia y ginecologiaFlorid papillomatosis of the nipple.
BMJ case reportsMammary Paget's Disease of the Male Breast: A Rare Case With an Unusual Immunohistochemical Profile.
International journal of surgical pathologyUnilateral nipple hyperkeratosis.
JAAD case reportsSurgical management of ductal carcinoma in situ of the breast: A large retrospective study from a single institution.
The breast journalDermoscopic 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 : JEADVIsolated invasive ductal carcinoma of the nipple-areolar complex: A rare occurrence yet to be reported in current literature.
The breast journalNipple and areola lesions: review of dermoscopy and reflectance confocal microscopy features.
Journal of the European Academy of Dermatology and Venereology : JEADVExtramammary Paget disease.
Dermatology online journal[The Diagnostic Problem of the Automated Breast Volume Scanner(ABUS) for Paget Disease-A Case Report].
Gan to kagaku ryoho. Cancer & chemotherapyExtramammary Paget Disease-A Challenging Case.
The American Journal of dermatopathologyOnline Information-Seeking About Potential Breast Cancer Symptoms: Capturing Online Behavior With an Internet Browsing Tracking Tool.
Journal of medical Internet researchIncidental 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[A Case of Invasive Ductal Carcinoma with Paget's Disease of the Breast].
Gan to kagaku ryoho. Cancer & chemotherapyPrognostic significance of mammary Paget's disease in Chinese women: a 10-year, population-based, matched cohort study.
OncoTargets and therapyMammary Paget's Disease of the Nipple: Relatively Common but Still Unknown to Many.
Korean journal of family medicineThe utility of nipple scraping in the diagnosis of Paget disease of the breast.
Diagnostic cytopathologyNipple and areola lesions: Dermoscopy and reflectance confocal microscopy features.
Journal of the American Academy of DermatologySkin diseases of the breast and nipple: Benign and malignant tumors.
Journal of the American Academy of DermatologySquamous Cell Carcinoma Arising within Verruca Vulgaris on the Nipple.
Case reports in dermatological medicineBilateral mammary Paget disease in a young adult female.
Radiology case reportsMammary Paget's disease without underlying malignancy of the breast.
Yeungnam University journal of medicineUnilateral nipple erosion with acantholysis.
International journal of dermatologyPigmented mammary Paget disease mimicking cutaneous melanoma.
International journal of dermatologySonographic visualization of nipple blood flow can help differentiate Paget disease from benign eczematous nipple lesions.
PloS onePractical consensus recommendatons for Paget's disease in breast cancer.
South Asian journal of cancerPaget's disease of the male breast: case report and a point of view from actual literature.
Il Giornale di chirurgia[Paget's disease of ectopic breast].
Annales de dermatologie et de venereologieInvasive Solid Papillary Carcinoma of the Nipple With Pagetoid Extension and Nodal Metastasis.
International journal of surgical pathologyPigmented Paget's disease of the nipple mistaken for melanoma in situ: a diagnostic pitfall for the unwary.
PathologyNipple adenoma in a 2-year-old boy.
Pediatric dermatologyNipple-Invasive Primary Carcinomas: Clinical, Imaging, and Pathologic Features of Breast Carcinomas Originating in the Nipple.
Archives of pathology & laboratory medicine[A Case of Paget-Type Recurrence 20 Years after Breast Conserving Surgery for Invasive Ductal Carcinoma].
Gan to kagaku ryoho. Cancer & chemotherapy[A Case of Adenoma of the Nipple Suspected of Breast Cancer].
Gan to kagaku ryoho. Cancer & chemotherapyPaget's Disease of the Breast: A dangerous imitator of eczema.
Sultan Qaboos University medical journalInvasive papillary breast cancer with Paget's disease: first reported association and literature review.
BMJ case reportsSolid papillary carcinoma of the breast with an associated Cytokeratin 7- negative Paget's disease of the nipple. Report of a first case.
The breast journalReflectance confocal microscopy of mammary Paget disease.
Dermatology practical & conceptualErythematous, friable nipple with loss of protrusion · history of breastfeeding · Dx?
The Journal of family practiceInvasion in breast lesions: the role of the epithelial-stroma barrier.
HistopathologyNipple-sparing mastectomy as treatment for patients with ductal carcinoma in situ: A 10-year follow-up study.
The breast journalPaget's Breast Disease: A Case Report and Review of the Literature.
Frontiers in surgeryMOHS micrographic surgery for treating erosive adenoma of the nipple: a case report and review of the literature.
International journal of dermatologyPyoderma Gangrenosum after Breast Mastectomy and Primary Rectus Abdominis Flap Reconstruction.
The Tokai journal of experimental and clinical medicine[Erosive lesion of the nipple].
The Pan African medical journalInvasive Paget's disease of the male nipple: a case report.
Journal of surgical case reportsClinical and Sonographic Features of Mammary Paget's Disease.
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeAn atypical presentation of Paget's Disease of the breast without nipple involvement: Case report and review of the literature.
Pathology, research and practiceDermoscopy as a Supportive Instrument in the Early Recognition of Erosive Adenomatosis of the Nipple and Mammary Paget's Disease.
Annals of dermatology[Hyperkeratotic lesion of the nipple and areola].
Presse medicale (Paris, France : 1983)A common cancer in an uncommon location: A case report of squamous cell carcinoma of the nipple.
International journal of surgery case reportsPaget disease of the male breast.
Dermatology online journal[Paget's disease of the nipple].
The Pan African medical journalOccurence 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...Glandular Paget's Disease of the Male Nipple.
Annals of dermatologyPaget's disease of the male breast in the 21st century: A systematic review.
Breast (Edinburgh, Scotland)An unusual case of mammary Pagets disease in a woman with psoriasis.
Journal of biological regulators and homeostatic agentsNipple 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 dermatologyPaget's Disease of Nipple in Male Breast with Cancer.
Journal of clinical and diagnostic research : JCDRChallenges in Diagnosis and Management of Paget's Disease of the Breast-a Retrospective Study.
The Indian journal of surgeryCytokeratin 7-negative mammary Paget's disease: A diagnostic pitfall.
Pathology, research and practicePaget's disease of the male breast: a case report.
Surgical case reportsMimics of Mammary Paget Disease: A Rogues' Gallery.
International journal of surgical pathologyDiagnosis and treatment of Paget's disease of the breast: an analysis of 72 cases.
International journal of clinical and experimental medicineSynchronous 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 medicinePigmented lesion of the nipple - a clinicopathological challenge.
Dermatology online journal[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 reproductionAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Existe um documento oficial para "Doença de Paget". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.
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.
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
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata