Exxcellence pearls: Paget disease of the Vulva

Question 0

How should patients be counseled regarding excision and margin status?

Answer 0

Up to 50% recurrence risk even with negative margins due to skip lesions. Requires long-term monitoring and repeat biopsies. May be associated with increased risk of breast, colon, lung, or gynecologic malignancy.

Question 1

How does Paget’s disease of the vulva typically present?

Answer 1

Women over 60 typically present with severe pruritus. On physical examination there is usually red, weeping lesions with a “cupcake frosting” eczematoid appearance.

Question 2

How can diagnosis be confirmed?

How is it treated?

Answer 2

Biopsy (read by a good vulvar pathologist)

Wide local excision