Treatment of vulvar Paget disease with topical imiquimod: a case report and review of the literature

Abstract

Background: Extramammary Paget's disease is a cutaneous neoplasm that presents as erythematous crusted patches and plaques reminiscent of contact dermatitis or inverse psoriasis that can be a challenge to treat in a tissue-sparing manner. The most commonly involved site for this rare disorder is the anogenital region. Surgery is considered as the gold standard therapy. In the last years, the topical use of imiquimod cream in the treatment of this condition has been reported.

Extramammary Paget's disease of the Vulva

“…Topical treatment using imiquimod 5% has been reported to produce remissions in small tumours but not in all 8 9. Other options include 3.5% bleomycin, 5-flurouracil, CO 2 laser and radiotherapy.…”

Staged marginal contoured and central excision technique in the surgical management of perianal Paget's disease

“…31,32 The use of imiquimod for EMPD has been described in recent case reports and small series of patients. 33 Some reports of patients with vulvar EMPD treated with local 5% imiquimod cream can show clinical and histologic remission within weeks of treatment. [34][35][36] No long-term results have been published to determine the recurrence rate of this treatment modality.…”

Surgical treatment of Paget’s disease of the vulva using Mohs micrographic surgery, followed by vulvar reconstruction using the “lotus petal” suprafascial flap

“…With respect to the EMPD prognosis in general, it is limited by the multifocal nature of EMPD [8] . Topical treatment using imiquimod 5% has been reported in the past few years with varying results, occasionally yielding remissions in small tumors and in recurrent cases [3,4,13] . However, there is a need for larger controlled trials to accurately determine the efficacy, as well as the safety of the imiquimod 5% cream [3,4] .…”

“…Topical treatment using imiquimod 5% has been reported in the past few years with varying results, occasionally yielding remissions in small tumors and in recurrent cases [3,4,13] . However, there is a need for larger controlled trials to accurately determine the efficacy, as well as the safety of the imiquimod 5% cream [3,4] . In this report, our team demonstrated the "lotus petal" suprafascial skin flap technique, as described by Ragoowansi et al [14] , which is a simple procedure that can be carried out immediately after a radical vulvectomy.…”

“…Although rare, it should be considered in the differential diagnosis of any chronic dermatitis of the perineum [1,2] . While the current gold standard for the treatment of vulvar EMPD is surgical excision, with Mohs micrographic surgery being the preferred technique, recent scientific publications have proposed the use of imiquimod 5% (applied topically) as an alternative for primary EMPD [3,4] . However, such case reports typically have limited follow-ups compared to classical surgical resection studies [5] .…”