• Philip R Cohen, Keith E Schulze, Jaime A Tschen, George W Hetherington, and Bruce R Nelson.
• Dermatologic Surgery Center of Houston, Houston, TX, USA.
• South. Med. J. 2006 Apr 1; 99 (4): 396-402.

AbstractExtramammary Paget disease is an uncommon cutaneous neoplasm that presents as erythematous plaques most frequently located in the anogenital region. Management of patients with extramammary Paget disease involves evaluation of the individual for: (1) a disease-associated, unsuspected, visceral malignancy and (2) secondary adenocarcinoma in the underlying dermis or regional lymph nodes. Several modalities, each with variable effectiveness, are available to treat the cutaneous component of the disease: electrodesiccation and curettage, laser surgery, aminolevulinic acid photodynamic therapy, radiotherapy, topical chemotherapy, and wide surgical excision. However, surgical excision using the Mohs micrographic technique is currently the modality of choice for treating the cutaneous lesions of extramammary Paget disease. Recently, a topical imidazoquinoline immunomodulator that induces cytokine production and stimulates the innate and cellular immune responses--imiquimod cream-has been used for the management of primary or relapsing extramammary Paget disease. Complete healing, without recurrence, of extramammary Paget disease in patients whose cutaneous lesions were treated topically with imiquimod 5% cream was observed. We describe a man with suprapubic extramammary Paget disease whose condition was primary and limited to his skin. Biopsy-confirmed complete resolution of his disease was observed after the topical application of imiquimod 5% cream 3 times per week (on alternate days) for 16 weeks. After reviewing the published reports of other patients with extramammary Paget disease whose disease was successfully treated with imiquimod cream, we suggest that topical imiquimod 5% cream-at least 3 times per week (with 1-2 d of nontreatment in between) for a minimum of 8 to 16 weeks--be considered as an initial treatment for primary cutaneous extramammary Paget disease. Surgical excision or an alternative therapeutic modality is recommended for patients whose extramammary Paget disease persists or recurs after treatment with topical imiquimod.

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