Metastatic Primary Extramammary Paget Disease: A Case Series

Extramammary Paget disease (EMPD) is an uncommon cutaneous malignancy that manifests with pruritic erythematous plaques within apocrine-rich areas such as the axillae and anogenital region. Dermal invasion is a known risk factor for metastasis, which is associated with poor outcomes. We present 2 cases of invasive EMPD on initial biopsy with rapid disease progression. The patients died secondary to metastatic EMPD without additional underlying malignancy. We review the literature and highlight key clinicopathologic features, management considerations, and the potential for rapid disease progression in cases of invasive EMPD.