A rare type of cancer, extramasmal Paget disease (EMPD) usually affects skin areas with apocrine sweat glands, such as the genital area, perianal region, and axilla. In this blog post, we provide a straightforward overview of EMPD, including its causes, symptoms, diagnosis, treatment, and prevention.
Causes
Extramammary Paget disease is not well understood, but it is believed to result from the malignant transformation of skin epithelial cells. Inflammation or irritation of the skin, genetic predisposition, and underlying conditions like hidradenitis suppurativa can all lead to EMPD.
Symptoms
Extramammary Paget disease may present with the following symptoms:
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Affected areas may appear red, scaly, or eczema-like, and may feel itchy or burning.
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A persistent rash or lesion that does not heal or responds poorly to topical treatment.
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The affected skin may become thickened, ulcerated, or crusted in some cases.
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When the lesion becomes ulcerated or infected, pain or discomfort may occur in the affected area.
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When ulcerated or traumatized, lesions may bleed or ooze bloody discharge.
Diagnosis
Extramammary Paget disease is typically diagnosed by:
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Examination of the affected skin to determine its appearance, location, and extent.
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Skin biopsy: A skin biopsy can be performed to obtain a tissue sample for histological examination, which can confirm the presence of Paget cells and rule out other skin conditions.
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In order to assess the extent of disease involvement and evaluate for underlying malignancy or metastasis, imaging tests such as ultrasound, magnetic resonance imaging, or computed tomography scans may be performed.
Treatment
The treatment of extramammary Paget disease depends on the extent of disease involvement, and may include the following:
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In order to completely remove a tumor, the affected skin and underlying tissues may be surgically removed.
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The Mohs Micrographic Surgery technique allows for precise removal of cancerous tissue while preserving healthy tissue surrounding it.
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As adjuvant therapy following surgical excision, topical therapies such as imiquimod or 5-fluorouracil (5-FU) may be used to treat superficial lesions.
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Patients who are not surgical candidates or who want to reduce the risk of local recurrence may be recommended radiation therapy.
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In advanced or metastatic disease that cannot be treated surgically or locally, systemic chemotherapy, targeted therapy, or immunotherapy may be considered.
Prevention
Extramammary Paget disease can be prevented primarily by minimizing risk factors and promoting skin health:
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Skin cancer and other dermatological conditions can be reduced by protecting the skin from excessive sun exposure.
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The best way to prevent skin inflammation or irritation is to practice good skin hygiene and avoid prolonged exposure to irritants or allergens.
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Regular self-examinations and routine skin checks by a dermatologist can help detect early signs of skin cancer.
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For any suspicious skin lesions or changes in the appearance of the skin, especially in high-risk areas such as the genital region or axilla, seek prompt medical attention.
This type of skin cancer primarily affects areas of the skin with apocrine sweat glands. Extramammary Paget disease is a rare type of skin cancer. Individuals affected by this condition can receive timely and appropriate care if they understand its causes, symptoms, diagnosis, treatment, and prevention strategies. You should consult a dermatologist or healthcare provider if you suspect that you have extramammary Paget disease or notice any changes to your skin.
