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Review
. 2023 Nov 30;15(23):5665.
doi: 10.3390/cancers15235665.

Topical Imiquimod in Primary Cutaneous Extramammary Paget's Disease: A Systematic Review

Fátima Mayo-Martínez  1 , Ruggero Moro  2   3 , David Millán-Esteban  1   2 , Elisa Ríos-Viñuela  2 , Iker Javier Bautista  4   5 , Eduardo Nagore  1 , Onofre Sanmartín  1 , Beatriz Llombart  1
Affiliations
Review

Topical Imiquimod in Primary Cutaneous Extramammary Paget's Disease: A Systematic Review

Fátima Mayo-Martínez et al. Cancers (Basel). .

Abstract

Extramammary Paget's disease (EMPD) is subclinical in extent and multifocal in nature. There is no global consensus for treatment, so its management represents a challenge in clinical practice. Therefore, we conducted a systematic review through the main electronic databases to assess the effectiveness of topical imiquimod in cutaneous EMPD and to discuss its management. Finally, 24 studies involving a total of 233 EMPD patients treated with topical imiquimod were selected. The topical imiquimod response rate was 67%, and the complete response (CR) rate was 48%. Patients were treated with a three-four times a week regimen in most cases, ranging between 2 to 52 weeks. In addition, imiquimod was applied as an adjunctive treatment in 21 patients, achieving a CR rate of 71%. Consequently, imiquimod therapy could achieve a good response ratio as a first-line treatment, as adjuvant and neo-adjuvant therapy, and as a treatment for recurrent disease. The heterogeneity between studies and the lack of a control arm made it impossible to conduct a meta-analysis. To improve the quality of evidence on EMPD, multicenter studies are essential to collect a larger number of patients and, consequently, obtain high-quality evidence to standardize treatment. The Prospero registration number is CRD42023447443.

Keywords: cutaneous Paget’s disease; extramammary Paget disease; extramammary Paget’s disease; imiquimod; perianal Paget’s disease; scrotal Paget’s disease; topical immunotherapy; treatment; vulvar Paget’s disease.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Histopathologic features of extramammary Paget’s disease. Paget’s cells proliferate within the epidermis mainly as isolated units, associating a nested pattern in some areas. Paget’s cells are large, with ample, pale, and finely granular cytoplasm and round and pleomorphic nuclei.
Figure 2
Figure 2
Extramammary Paget’s disease of the vulva treated with topical imiquimod: (a) Before treatment; (b) After 9 weeks (27 applications) of topical imiquimod; (c) After 15 weeks (47 applications) of topical imiquimod; (d) Six months after the end of topical imiquimod therapy.
Figure 3
Figure 3
PRISMA flow chart. Provides details on the literature search.
See this image and copyright information in PMC

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