Extramammary Paget's disease recurring on the skin of the lower abdomen and entire right leg two and half years after simple vulvectomy for minimally invasive Paget's disease of the vulva.

We encountered an unusual case of extramammary Paget's disease (EMPD) recurring on the skin of the lower abdomen and entire right leg two and a half years after simple vulvectomy for minimally invasive Paget's disease of the vulva. Histologic examination of the skin metastases demonstrated that the proliferation of Paget cells was confined to the dermis, most of them were located in lymphatic vascular spaces. This case confirms that minimally invasive Paget's disease of the vulva may sometimes be an aggressive disease. We consider that lymphatic metastases already existed in this patient at the time of initial surgery; thus, the recurrence of EMPD on extragenital skin sites may have been prevented if initial treatment would have included radical vulvectomy and bilateral groin lymph node dissection instead of simple vulvectomy without groin lymph node dissection.