Abstract

Extramammary Paget's Disease is a rare disease. There is a close correlation with anorrectal carcinoma when the disease is perianal. It is also associated with internal malignancies and bad prognosis. The authors describe a case of extramammary Paget's Disease in perineo-inguinoscrotal region of a 59-year-old-white man, who had a hyperpigmented and pruritic lesion of approximately 6 cm of diameter which has increased and was presenting minimum bleeding. Staging tests were normal, without evidence of metastasis. The anatomopathological test of the lesion revealed na aspect compatible with extramammary Paget's Disease. The patient was submmited to an enlarged excision of the lesion and thin skin graft. The aspects related to epidemiology, pathology, staging, prognosis and treatment of this neoplasia are discussed in this article.

Key words: Paget's Disease, extramammary, skin, malignant neoplasms.