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Extramammary Paget disease (EMPD) of the vulva is a rare, slow-growing intraepithelial adenocarcinoma, typically arising in postmenopausal women.

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It involves the labia majora in >90% of cases and can be primary (cutaneous origin) or secondary (associated with an underlying malignancy such as Bartholin gland or anorectal adenocarcinoma).

Pathophysiology

• EMPD is analogous to mammary Paget disease, but affects apocrine gland–bearing skin.
• It involves intraepithelial infiltration by malignant glandular cells (Paget cells) without dermal invasion in early stages.

Epidemiology

• Rare: accounts for <1% of vulvar cancers
• Typically seen in elderly women (>60 years)
• May coexist with or precede underlying adenocarcinoma (15–30% cases)

Clinical Features

• Chronic pruritic, erythematous, velvety plaque or eczematous lesion on the labia majora
• May resemble eczema or fungal infection; often misdiagnosed initially
• Lesion may be:
  • Well-defined or infiltrative
  • Red, scaly, or ulcerated
  • Sometimes pigmented or nodular
• Recurrent lesions are common after excision

Diagnosis

• Biopsy is essential:
  • Shows large, pale-staining Paget cells within the epidermis
  • May require multiple samples to assess depth/invasion