What Is Extramammary Paget Disease and How Is It Treated?

Written by Medicover Team and Medically Reviewed by Dr Anusha Nagral Reddy , Dermatologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Extramammary Paget's disease is a rare type of skin cancer that usually affects areas containing apocrine glands, most commonly the genital, perianal, or groin regions. It can also occur in areas such as the armpits.

This condition can cause persistent skin changes, discomfort, and emotional distress. Early evaluation and appropriate treatment are important because extramammary Paget's disease may sometimes be associated with an underlying cancer in nearby organs.

What Are the Types of Extramammary Paget's Disease?

Extramammary Paget's disease can occur in different locations, most often affecting the anogenital region. The clinical appearance and underlying cause may vary depending on the affected area.

Identifying the location and extent of the disease helps doctors determine whether it is limited to the skin or associated with an underlying malignancy and guides treatment planning.

Vulvar Extramammary Paget's Disease: A rare form that affects the skin of the vulva and surrounding genital area.
Anogenital Extramammary Paget's Disease: Affects the skin around the anus and genital region.
Perianal Extramammary Paget's Disease: Involves the skin surrounding the anus and may sometimes be associated with an underlying gastrointestinal or anal malignancy.
Axillary Extramammary Paget's Disease: A rare form that affects the skin of the armpit.
Primary Extramammary Paget's Disease: The Paget cells originate within the skin and are not caused by an underlying cancer in another organ.
Secondary Extramammary Paget's Disease: Occurs when Paget cells spread to the skin from an underlying malignancy in a nearby or related organ.

What Are the Symptoms of Extramammary Paget's Disease?

Extramammary Paget's disease commonly causes persistent changes in the skin of the genital, perianal, groin, or armpit areas. These changes can resemble eczema, dermatitis, fungal infections, or other common skin conditions.

Because the condition can remain undiagnosed for a long time, persistent or recurrent skin changes that do not improve with usual treatment should be evaluated by a healthcare professional.

Persistent redness or a red rash
Itching or burning of the affected skin
Scaling, crusting, or thickening of the skin
Skin discoloration or changes in skin texture
Moist or oozing skin lesions
Non-healing sores or ulceration
Pain or tenderness in the affected area
Bleeding from the affected skin

What Are the Causes of Extramammary Paget's Disease?

The exact cause of extramammary Paget's disease is not fully understood. It develops when abnormal glandular cells, known as Paget cells, grow within the skin and form a cancerous lesion.

In some people, the abnormal cells originate in the skin itself, while in others they may be associated with an underlying cancer in nearby organs. Genetic changes within the abnormal cells are believed to contribute to their uncontrolled growth.

Abnormal growth of Paget cells within the skin
Genetic changes affecting the growth and behavior of skin cells
Underlying adenocarcinoma or another malignancy in some cases
Association with cancers of nearby organs, depending on the location of the lesion

When to See a Doctor?

Consult a dermatologist if you develop persistent itching, redness, scaling, thickening, or a non-healing lesion in the genital, perianal, groin, or armpit area. A dermatologist can examine the skin and arrange a biopsy if extramammary Paget's disease is suspected.

If the diagnosis is confirmed, a surgical oncologist or other cancer specialist may be involved in treatment, particularly when the disease is extensive or associated with an underlying malignancy.

Persistent itching, redness, or rash in the genital or nearby areas
Skin thickening, scaling, crusting, or a non-healing lesion
Persistent pain, burning, or discomfort
Changes in the appearance or texture of the affected skin
Skin symptoms that do not improve with routine treatment

Get medical help promptly if:

Skin lesions begin bleeding or ulcerating
There is rapidly worsening pain or swelling
The affected area develops signs of infection
Skin changes spread or worsen despite treatment

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How Is Extramammary Paget's Disease Diagnosed?

Extramammary Paget's disease is diagnosed through a clinical examination and skin biopsy. Because its appearance can resemble several common skin conditions, a biopsy is important for confirming the presence of Paget cells.

Additional investigations may be recommended after diagnosis to determine how deeply the disease has affected the skin and to check for an underlying malignancy when clinically indicated.

Physical Examination: Examination of the affected skin and assessment of the size, appearance, and location of the lesion.
Skin Biopsy: A biopsy of the affected skin is used to confirm the diagnosis and identify Paget cells.
Immunohistochemistry: Special laboratory staining may help confirm the diagnosis and distinguish extramammary Paget's disease from other skin conditions.
Imaging Tests: CT, MRI, or other imaging studies may be used when an underlying malignancy or deeper disease involvement is suspected.
Additional Cancer Evaluation: Depending on the location and clinical findings, doctors may recommend investigations to look for an associated cancer in nearby organs.

What Are the Treatment Options for Extramammary Paget's Disease?

Treatment for extramammary Paget's disease depends on the location, size, depth, and extent of the lesion, as well as whether an underlying cancer is present. Surgery is commonly used when the disease is localized and can be completely removed.

The treatment plan is individualized and may involve dermatologists, surgeons, oncologists, and other specialists when necessary. Additional treatment may be considered when the disease is extensive, recurrent, or associated with an underlying malignancy.

Surgical Excision: Surgical removal of the affected skin is a common treatment for localized extramammary Paget's disease. The surgeon aims to remove the lesion with clear margins.
Mohs Micrographic Surgery: Mohs surgery may be considered for selected lesions in areas where preserving healthy tissue is important.
Radiation Therapy: Radiation therapy may be considered when surgery is not suitable, when the disease cannot be completely removed, or in selected recurrent cases.
Topical Treatments: Medicines such as topical imiquimod may be considered in selected superficial cases under specialist supervision.
Treatment of Underlying Cancer: If extramammary Paget's disease is associated with an underlying malignancy, treatment is directed at that cancer according to its type and stage.
Follow-up and Monitoring: Regular follow-up examinations are important because extramammary Paget's disease can recur after treatment.

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What Are the Risk Factors for Extramammary Paget's Disease?

Extramammary Paget's disease is rare, and its exact risk factors are not fully established. It is more commonly diagnosed in older adults and may occur in areas containing apocrine glands.

A history of certain underlying malignancies may also be relevant, particularly in secondary extramammary Paget's disease. Persistent or unexplained skin changes should therefore be evaluated rather than assumed to be a routine skin condition.

Older age
Persistent or chronic skin inflammation in the affected area
Underlying malignancy in some individuals
Conditions affecting areas containing apocrine glands

Frequently Asked Questions

1. What is Extramammary Paget disease?

Extramammary Paget disease is a rare skin cancer that usually affects areas with apocrine glands, such as the vulva, scrotum, penis, or around the anus. It often appears as a persistent red, scaly, itchy, or irritated patch.

2. What are the symptoms of Extramammary Paget disease?

Symptoms may include persistent itching, burning, redness, scaling, crusting, oozing, or a thickened skin patch. The affected area may resemble eczema or dermatitis.

3. What causes Extramammary Paget disease?

The exact cause is not fully understood. It develops when Paget cells grow abnormally in the skin and may sometimes be associated with an underlying cancer in nearby tissues.

4. How is Extramammary Paget disease diagnosed?

A skin biopsy is needed to confirm the diagnosis. Additional tests may be recommended to check for an underlying cancer, depending on the affected area and biopsy findings.

5. How is Extramammary Paget disease treated?

Treatment usually involves surgical removal of the affected skin, often with margin assessment. Depending on the extent and location, other treatments such as Mohs surgery, radiation therapy, or topical medicines may be considered.