01OverviewDefinition, clinical context and the essential points that orientate the chapter.
Paget disease reflects malignant epithelial cells within the nipple epidermis, commonly connected to carcinoma in the ducts beneath the nipple or elsewhere in the breast. Disruption of the normal epidermal barrier produces a red scaly or eroded surface that can resemble benign dermatitis. Its danger lies in treating the visible skin change without establishing why malignant cells reached the epidermis or whether invasive disease is present deeper in the breast.
Diagnosis therefore has two linked tasks: sample the abnormal epidermis and map the underlying breast. A punch or wedge biopsy can demonstrate Paget cells, while mammography and ultrasound assess calcification, a retroareolar lesion and nodes. MRI is selected when conventional assessment does not define a management-relevant extent, rather than used automatically. Surgery ranges from excision of the nipple-areolar complex with breast conservation to mastectomy according to distribution, invasive components, achievable margins and patient preference.
Key points
- Paget disease of the nipple is intraepidermal spread of malignant glandular cells and is usually associated with underlying ductal carcinoma in situ or invasive breast carcinoma.
- The classical lesion starts on one nipple and may extend to the areola, causing erythema, scaling, crusting, erosion, itch, pain, bleeding or flattening.
- Bilateral areolar dermatitis that spares the nipple and promptly improves with appropriate skin care is more typical of eczema; persistence or nipple involvement overrides that reassurance.
- Examine both breasts and nodes, obtain age-appropriate diagnostic mammography with targeted ultrasound, and biopsy the abnormal nipple skin when Paget disease remains possible.
- A negative mammogram does not exclude Paget disease or an underlying small malignancy. Clinical-pathological concordance and additional imaging are considered when the extent remains unresolved.
- For disease assessed as localised, NICE offers breast-conserving surgery removing the nipple-areolar complex as an alternative to mastectomy, with oncoplastic repair discussed for cosmesis.
02AetiologyUnderlying causes, associations and risk factors, with why each one matters.
Underlying ductal neoplasia
Most cases accompany ductal carcinoma in situ or invasive ductal carcinoma whose malignant cells involve the nipple epidermis.
Rare isolated epidermal disease
A smaller group has Paget cells confined to the nipple region without a detectable deeper tumour on complete assessment.
03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.
- 1Epidermal colonisation
Malignant glandular cells spread into nipple epidermis, disrupting keratinocytes and the normal barrier and producing scale, crust and erosion.
- 2Duct-to-nipple connection
Paget cells commonly track through lactiferous ducts from underlying carcinoma, linking the visible skin lesion to deeper breast disease.
- 3Inflammatory surface reaction
Epidermal injury provokes erythema, itch, serous ooze and ulceration, which creates the clinical resemblance to eczema or infection.
04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.
Scaling, crusting, erythema or erosion begins on the nipple itself and may spread outward; progressive unilateral change is more concerning than symmetrical irritation.
Itching, burning, bleeding, ulceration, discharge, flattening or new inversion can accompany the epidermal lesion and strengthen the need for tissue diagnosis.
A palpable mass or suspicious node increases the likelihood of invasive carcinoma and changes axillary and systemic planning.
Benign eczema often affects both sides, may spare the nipple and improves with appropriate topical care; failure to settle or direct nipple involvement requires reassessment.
05InvestigationsWhat to request, why it matters and how to interpret it.
Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.
- 01
Nipple skin punch or wedge biopsyFirst step - Why
- Demonstrate malignant Paget cells within the epidermis and distinguish cancer from inflammatory dermatoses.
- Interpretation and limitations
- Adequate tissue should include the active abnormal edge and epidermis. A superficial or non-representative negative sample cannot overrule a persistent clinically suspicious lesion.
- 02
Diagnostic mammography - Why
- Look for retroareolar calcification, a mass, distortion or more extensive ductal disease in both breasts.
- Interpretation and limitations
- An abnormality guides image biopsy and estimates extent. A normal mammogram does not exclude epidermal Paget disease or small underlying carcinoma.
- 03
Targeted breast and axillary ultrasound - Why
- Assess the retroareolar tissue, any palpable lesion and morphologically abnormal regional nodes.
- Interpretation and limitations
- A solid breast target needs core biopsy; an abnormal node is sampled if the result will alter staging and treatment.
- 04
Selected contrast-enhanced MRI - Why
- Clarify occult or uncertain extent when conventional imaging does not explain biopsy-proven Paget disease and the result could alter surgery.
- Interpretation and limitations
- Additional enhancement must be correlated and sampled where feasible before escalating from conservation to mastectomy.
06Differential diagnosisRealistic alternatives and the features that help distinguish them.
Nipple eczema
Dermatitis may scale and itch but is often bilateral, less nipple-centred and responsive to appropriate skin treatment.
Contact dermatitis
Soaps, adhesives, fabrics or topical products can inflame nipple-areolar skin; exposure history and resolution after avoidance support the diagnosis.
Nipple adenoma
A benign ductal proliferation can cause erosion, discharge or a nipple nodule and requires tissue distinction from Paget disease.
Melanoma or squamous malignancy
Pigmented or ulcerated nipple lesions may represent another skin cancer; biopsy morphology and immunohistochemistry determine lineage.
07ManagementImmediate care, first-line treatment, alternatives and escalation.
01Persistent nipple lesion pathwayBiopsy the skin and map the breastFirst stepA 63-year-old has four months of unilateral nipple crusting and bleeding that did not resolve with a topical steroid; no discrete mass is felt.+
- 1Document whether change began on the nipple, its duration, discharge and prior treatment; inspect both nipple-areolar complexes and examine breast and regional nodes.
- 2Arrange diagnostic mammography and targeted retroareolar ultrasound rather than prescribing another empirical dermatological course.
- 3Obtain an adequately deep punch or wedge biopsy from representative nipple skin, coordinating sampling so pathology can assess the epidermal process.
- 4If Paget disease is confirmed but conventional imaging is non-explanatory, ask whether selected MRI will resolve extent and change a realistic surgical choice.
- 5At the multidisciplinary meeting, integrate skin histology, any underlying core, imaging extent and axillary findings; give the patient a documented treatment and reconstruction discussion.
02Localised disease pathwayOffer a breast-conserving optionPaget disease and limited central DCIS are confirmed with no separate lesion and conservation can achieve clear margins.+
- 1Explain that localised disease can be treated by breast-conserving excision that removes the nipple-areolar complex, rather than assuming mastectomy is compulsory.
- 2Discuss the expected change in nipple sensation and appearance, oncoplastic repair, margin uncertainty and the usual role of postoperative radiotherapy after conservation.
- 3Plan axillary surgery according to whether invasion is present and whether the operation is conservation or mastectomy for DCIS.
- 4Review final pathology for invasive disease, DCIS extent and radial margins before completing adjuvant recommendations.
03Non-representative biopsy pathwayDo not let a weak sample close the caseA superficial shave is reported as non-specific dermatitis, but the unilateral nipple remains ulcerated and the mammogram shows retroareolar calcification.+
- 1Ask pathology whether the specimen contained adequate full epidermis from the active lesion and whether the report explains the clinical appearance.
- 2Core the mammographic target using image guidance and obtain repeat representative nipple skin tissue.
- 3Avoid prolonged steroid treatment while clinical, imaging and pathological findings remain discordant.
- 4EscalationEscalate the complete case to breast multidisciplinary review and proceed only when both the skin lesion and underlying target have been explained.
08ComplicationsImportant consequences, why they occur and why they matter clinically.
Underlying invasive carcinoma
Stromal invasion adds regional nodal and distant metastatic risk and changes axillary and systemic treatment planning.
Progressive ulceration
Untreated epidermal disease can cause painful erosion, bleeding, secondary infection and destruction of the nipple-areolar complex.
Local recurrence
Unrecognised multifocal or extensive ductal disease can recur after inadequate central excision, especially if margins remain involved.
Surgical body-image impact
Removal of the nipple-areolar complex changes breast identity, sensation and symmetry even when the remaining breast is conserved.
09Monitoring and follow-upTreatment response, safety checks and longer-term review.
- Before definitive treatment, confirm that nipple histology and each radiological target have been sampled or explicitly accounted for at multidisciplinary review.
- After breast conservation, check final invasive and DCIS extent, radial margins and radiotherapy recommendation; nipple-surface clearance alone is not the entire pathology endpoint.
- After surgery, review wound healing, infection, seroma, sensation, body-image concerns and access to oncoplastic or prosthetic support.
- Use annual mammography for five years after breast cancer treatment under NICE and assess any new skin, nipple, breast or axillary symptom promptly between visits.
10Special situationsVariants, exceptions and circumstances that change the usual approach.
The nipple origin matters
Paget disease typically begins on the nipple and spreads to areola; eczema more often involves areolar skin and may spare the nipple.
Skin biopsy is indispensable
Imaging can map an underlying carcinoma but cannot replace histological confirmation of a persistent malignant-appearing epidermal lesion.
Normal imaging is incomplete reassurance
The epidermal tumour may be present without an obvious mammographic mass, so persistent suspicious skin change still requires representative biopsy.
Localised disease permits conservation
Central breast conservation removes the nipple-areolar complex, not merely the crusted surface, and is paired with margin and radiotherapy planning.
11Common pitfallsFrequent interpretation and management errors.
- 01
Repeatedly prescribing topical steroid for a unilateral nipple-centred lesion can suppress inflammation temporarily while the underlying malignancy progresses.
- 02
Assuming that absence of a palpable breast mass excludes Paget disease ignores its common association with non-palpable DCIS.
- 03
Accepting a superficial non-representative skin specimen as definitive creates false reassurance when clinical suspicion and imaging remain abnormal.
- 04
Calling every Paget diagnosis an automatic mastectomy denies the NICE-supported conservation option for appropriately assessed localised disease.