[Extramammary Paget's disease: clinical and histologic documentation of 2 forms]

There are two forms of extramammary Paget's disease in respect of clinic, therapy and prognosis. In most cases (about 95%) extramammary Paget's disease is an intraepidermal manifestation of a cutaneous adenocarcinoma. After horizontal spreading within the epidermis, malignant growth continues along the adjacent adnexal structures with dermal spreading and subsequent metastases. In rare cases (approx. 5%), extramammary Paget's disease is an epidermal manifestation of an adenocarcinoma primarily located in the intestinal or urogenital tract. Further diagnostic and therapeutical procedures depend upon the type of extramammary Paget's disease.