Extramammary Paget's disease with distant skin metastasis

We report a 68-year-old Japanese man with invasive extramammary Paget's disease (EPD) which started in the left inguinal area and enlarged to include the scrotal skin, penis, and pubic area, resulting in lymph edema of the entire left leg. Histopathological examination of one nodule revealed an intraepidermal and intradermal arrangement of solid tumor cells with characteristic pale cytoplasm. The tumor was found to have disseminated to regional and systemic lymph nodes, lungs, bones, and the left adrenal gland. In addition, the patient had a metastatic cutaneous nodule on the scalp. Histology of the skin metastasis showed that the proliferation of Paget cells was confined to the dermis. This type of metastatic spread of EPD is very rare; only two other apparent cases in Japan have been reported.