Extramammary Paget's Disease

A red, scaly, intraepithelial tumor of the vulva that can mimic dermatoses. Primary extramammary Paget's is an apocrine adenocarcinoma that arises in the epidermis or epidermal appendages. Secondary extramammary Paget's is a visceral carcinoma from rectal, anal or other tissue that is epidermotropic to the skin.

Clinical: Patients present with a chronic, itchy rash that has been non-responsive to topical steroids yet looks eczematous red and scaly. Secondary changes with erosions and mild crusting.

Diagnosis: Biopsy. Management: Surgery Investigation for underlying cancer in the genitourinary or perianal or anal canal. Intraepithelial Paget's disease rarely progresses to invasive carcinoma.