2 cm firm violaceous plaque with surrounding macular hyperpigmentation was present at the previous extramammary Paget disease site (Fig.
There has been no recurrence of the patient's cutaneous extramammary Paget disease on the follow-up examinations during the subsequent 9 months after treatment was discontinued.
Extramammary Paget disease may occur in four clinical settings: (1) as a primary--isolated and limited--cutaneous form with only intraepidermal involvement, (2) as a primary intraepidermal disease with secondary adenocarcinoma present in the underlying dermis or regional lymph nodes, (3) as a secondary skin disease arising from an underlying adnexal carcinoma, and (4) as a cutaneous (epidermal or combined epidermal and dermal) disease associated with internal malignancy.
The treatment of choice for extramammary Paget disease is surgical excision--optimally incorporating the Mohs micrographic technique.
7) However, the specific mechanism of action whereby imiquimod promotes tumor eradication in extramammary Paget disease remains to be determined.
Successful management of previously untreated (8,13,15,16) (also current report) or recurrent (8-12,14,17) extramammary Paget disease after topical treatment with imiquimod 5% cream, including our patient, has been reported in 9 individuals, 8 men and 1 woman (Table) (8-17).
Extramammary Paget disease was primary and limited to the epidermis in 7 of the patients, (8,13,14-17) (also current report).