Depigmentation of the vulvar area: Is it an alarming sign?

A 34-year-old female patient presented with recurrent bilateral hypopigmented macules on the labia majora. The lesions were treated with topical steroids, which led to mild improvement, but erosive plaques developed after discontinuing the treatment. Histopathological findings were compatible with extramammary Paget disease (EMPD), which was treated with radical vulvectomy with no recurrence in the next months of follow-up.