Paget’s disease: information for patients

Cutaneous Paget’s disease is a rare condition that can affect the nipple and areola, or appear outside the breast in areas such as the vulva, penis, scrotum, groin, perianal region, or armpit. Its significance lies in the fact that it can resemble eczema, dermatitis, or a superficial infection, but in some cases, it corresponds to a proliferation of malignant cells in the epidermis. In the breast, it is usually associated with underlying breast cancer; in the extramammary form, it can be a primary skin cancer or related to tumors of nearby or internal organs.

Summary in 60 seconds

• Cutaneous Paget’s disease is a rare form of cancer that manifests in the skin.
• It can appear on the nipple and areola, or in genital, inguinal, perianal or axillary areas.
• It may resemble eczema, with redness, scaling, crusting, itching, or burning.
• An important sign is persistence: it does not improve, it reappears, or it progresses despite usual treatments.
• The nipple may be accompanied by discharge, bleeding, retraction, or a lump in the breast.
• In genital or perianal areas, it can cause chronic itching, moist plaques, erosions, or bleeding.
• The diagnosis is not confirmed by appearance alone: it requires a skin biopsy.
• In Paget’s disease of the nipple, the breast is also studied with imaging.
• In the extramammary form, studies may be required to rule out associated tumors.
• Seeking medical advice early allows for confirmation of the diagnosis and safer management planning.

What is Paget’s disease?

Paget’s disease of the skin is a proliferation of abnormal cells within the epidermis, the outermost layer of the skin. There are two main forms.

Paget’s disease of the breast affects the nipple and often the areola. It is a rare presentation of breast cancer and accounts for a small proportion of all breast cancer cases. It may be associated with ductal carcinoma in situ or invasive carcinoma in the same breast. (Karakas 2011 , National Cancer Institute 2025)

Extramammary Paget’s disease occurs outside the breast, primarily in areas with apocrine glands, such as the vulva, perianal region, penis, scrotum, groin, or armpit. It is quite rare, usually progresses slowly, and can be mistaken for eczema, psoriasis, fungal infection, or chronic irritation for months. (British Association of Dermatologists 2021 , DermNet NZ 2020)

It should not be confused with Paget’s disease of bone , which is a bone remodeling disorder and belongs to another medical field.

Why does it appear?

In Paget’s disease of the nipple, one accepted explanation is that malignant cells originating in the mammary ducts migrate to the skin of the nipple and areola. Therefore, a persistent skin lesion on the nipple can be the visible manifestation of an underlying breast disease. Yasir 2023

In extramammary Paget’s disease, the origin can be primary , when it arises in the skin itself, or secondary , when it is related to a tumor in a nearby or internal organ. This does not mean that everyone with extramammary Paget’s disease has internal cancer, but it does justify a systematic evaluation based on the lesion’s location. Kibbi 2022

It is not caused by poor hygiene, casual contact, or contagion. Nor should any irritation in these areas be interpreted as Paget’s disease. The clinical key is the combination of location, persistence, symptoms, lack of response to standard treatments, and confirmation by biopsy.

What does it look like and what symptoms does it cause?

Paget’s disease can resemble eczema. This similarity explains the importance of a proper specialist evaluation.

It can produce the following on the nipple and areola:

• reddening;
• peeling;
• thick, cracked, or scabbed skin;
• itching, burning, tingling or pain;
• yellowish, clear or bloody secretion;
• nipple flattening or retraction;
• erosion, wound or bleeding;
• a lump in the breast or armpit, although it is not always present.

In its extramammary form, it may appear as a reddish, pinkish, brownish, whitish, or hyperpigmented plaque, with scaling, oozing, crusting, erosions, or thickening. It can cause intense itching, burning, pain, persistent irritation, bleeding, or a chronic wound sensation. British Association of Dermatologists 2021

In dark skin, the lesion does not always appear red. It may present as a darker, grayish, brownish plaque, or with mixed pigmentation changes.

What can you do today?

If you have a persistent lesion on the nipple, areola, vulva, penis, scrotum, groin, armpit, or perianal area, it is best to consult a specialist doctor as soon as possible and avoid measures that may irritate or delay diagnosis.

You can do the following:

• Stop using obvious irritants, such as perfumes, harsh deodorants, strong soaps, or perfumed intimate products.
• Use mild cleaners and avoid excessive friction.
• Do not scratch, pick at scabs, or manipulate the lesion.
• Take photos in good light every 1–2 weeks if you are waiting for a date.
• Note when the injury started and what treatments you have used.
• Consult again if you have already been treated for eczema, fungus or irritation and the lesion persists.
• In cases of nipple injury, seek medical evaluation if the change is unilateral, progressive, or accompanied by discharge, bleeding, retraction, or a lump.

The priority is not to panic, but to confirm what is happening. A persistent injury deserves diagnosis, not repeated treatments without reassessment.

What NOT to do?

It is not advisable to assume that every scaly or irritated lesion is common eczema, especially if it affects the nipple, areola, vulva, penis, scrotum, groin, or perianal region.

Avoid:

• Using topical corticosteroids for weeks or months without medical follow-up.
• Repeating antifungals without confirming a fungal infection.
• Apply alcohol, lemon, baking soda, essential oils, or homemade mixtures.
• Burn, scrape or remove scabs.
• Hiding genital or perianal injuries out of shame.
• Wait until intense pain appears before seeking medical advice.
• Relying solely on partial improvement if the injury returns or continues to grow.

In extramammary Paget’s disease, several clinical sources recommend suspecting it when a chronic anogenital plaque does not respond to usual treatment for dermatitis or superficial infection (Primary Care Dermatology Society 2022 ).

An eczema-like lesion on the nipple that persists or recurs deserves medical evaluation, not just more creams.

When should I consult?

Consult a dermatologist as soon as possible if any of these signs appear:

• Nipple or areola lesion that does not improve or recurs.
• Unilateral nipple change: redness, crusting, scaling, wound or retraction.
• Nipple discharge, especially if it contains blood.
• Lump in the breast or armpit.
• Persistent genital, inguinal, axillary or perianal plaque.
• Itching, burning, or chronic pain in the intimate area without a clear cause.
• Bleeding, ulceration, persistent moisture, or repeated crusting.
• A lesion treated as eczema or a fungal infection that does not improve.
• Progressive growth, change of color, or appearance of nodules.

What does a dermatologist usually do?

The dermatologist begins with a detailed medical history: duration of symptoms, previous treatments, response to creams, personal and family history, breast or gynecological studies, and recent changes in the area.

Next, examine the skin. You may use dermatoscopy, a tool that magnifies the image of the lesion and helps identify suspicious areas. Dermatoscopy does not replace a biopsy, but it can guide the evaluation.

The decisive step is usually a skin biopsy . This can be a punch, incisional, shave, or wedge biopsy, depending on the area, size, and suspected depth. The diagnosis is confirmed with histopathological examination and, in many cases, with immunohistochemistry.

In Paget’s disease of the nipple, in addition to a skin biopsy, the breast is examined with mammography, ultrasound, or magnetic resonance imaging, depending on the case. If a breast lesion is identified, a biopsy may also be required. (National Cancer Institute 2025 , Song 2015)

In extramammary Paget’s disease, the medical team may order additional studies depending on the location: gynecological evaluation, colonoscopy if the lesion is perianal, urinary studies if it is near the urethra, prostate evaluation in men, or imaging studies when deemed necessary. Kibbi 2022

Treatment is individualized. In many cases, it includes surgery to remove the lesion. In extramammary disease, wide local excision, Mohs micrographic surgery, radiotherapy, imiquimod, or other alternatives may be considered in selected situations. The choice depends on the extent, location, depth, margins, comorbidities, and presence or absence of associated disease (Shah 2024 ).

Frequently Asked Questions

Is Paget’s disease the same as eczema?

• No. It may look like eczema, but it’s not the same. Eczema is an inflammatory skin condition. Paget’s disease is a proliferation of malignant cells in the epidermis. That’s why a persistent lesion should be evaluated.

Does all nipple peeling mean cancer?

• No. Nipple peeling can be caused by irritation, contact dermatitis, eczema, friction, breastfeeding, psoriasis, or infection. However, if it is unilateral, persistent, recurring, or accompanied by discharge, bleeding, retraction, or a lump, a doctor should be consulted.

Can it affect men?

• Yes. It is less common, but it can affect men, both on the nipple and in genital or perianal areas.

How is the diagnosis confirmed?

• With a skin biopsy. The clinical appearance can be indicative, but it is not enough to confirm or rule out the disease.

Is extramammary Paget’s disease always associated with internal cancer?

• Not always. It can be a primary skin infection. Even so, in some patients it may be related to internal tumors or tumors in nearby organs, so the doctor may order further tests.

Is surgery always the treatment?

• Surgery is a common option, but it is not the only one in all cases. Treatment depends on the location, extent, depth, age, comorbidities, surgical margins, and presence of associated disease.

Can I return after the treatment?

• Yes. Extramammary Paget’s disease can recur, even after treatment. That’s why long-term follow-up is an important part of management.

Bibliography and sources

• National Cancer Institute. Paget disease of the breast. Bethesda: National Cancer Institute; 2025. https://www.cancer.gov/types/breast/breast-cancer-types/paget-disease-breast
• National Health Service. Paget’s disease of the nipple. London: NHS; 2023. https://www.nhs.uk/conditions/pagets-disease-nipple/
• British Association of Dermatologists. Extra-mammary Paget disease. London: British Association of Dermatologists; 2021. https://www.bad.org.uk/pils/extra-mammary-pagets-disease/
• DermNet NZ. Extramammary Paget disease of skin. Hamilton: DermNet NZ; 2020. https://dermnetnz.org/topics/extramammary-paget-disease
• Primary Care Dermatology Society. Extramammary Paget disease of skin. London: Primary Care Dermatology Society; 2022. https://www.pcds.org.uk/clinical-guidance/extra-mammary-pagets-disease
• Karakas C. Paget’s disease of the breast. J Carcinog. 2011;10:31. https://pmc.ncbi.nlm.nih.gov/articles/PMC3263015/
• Song Q, Zhang X, Yu Y, Chen J, Li S, Li J, et al. Diagnosis and treatment of Paget’s disease of the breast: an overview. Onco Targets Ther. 2015;8:1955-1961. https://pmc.ncbi.nlm.nih.gov/articles/PMC4694522/
• Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, et al. Evidence-based clinical practice guidelines for extramammary Paget disease. JAMA Oncol. 2022;8(4):618-628. https://pubmed.ncbi.nlm.nih.gov/35050310/
• Yasir M, Khan M, Lotfollahzadeh S. Mammary Paget disease. In: StatPearls. Treasure Island: StatPearls Publishing; 2023. https://www.ncbi.nlm.nih.gov/books/NBK563228/
• McDaniel B, Brown F, Crane JS. Extramammary Paget disease. In: StatPearls. Treasure Island: StatPearls Publishing; 2023. https://www.ncbi.nlm.nih.gov/books/NBK493224/
• Markarian S, Holmes DR. Mammary Paget’s disease: an update. Cancers. 2022;14(10):2422. https://pmc.ncbi.nlm.nih.gov/articles/PMC9139704/
• Shah RR, Fujisawa Y, Takahashi A, et al. Extramammary Paget disease. Part II: evidence-based management. J Am Acad Dermatol. 2024. Available from: https://www.jaad.org/article/S0190-9622(24)00579-6/abstract