Extramammary Paget disease - clinical appearance, pathogenesis, management

Extramammary Paget disease is a rare malignant neoplasm. With regard to the pathogenesis, two prognostically different forms can be distinguished. The primary form of extramammary Paget disease is an in situ carcinoma of the apocrine gland ducts. In contrast, the secondary form is characterized by an intraepithelial spread due to an underlying carcinoma of the skin or other organ systems. Extramammary Paget disease occurs in older patients. The predilection sites include the entire anogenital skin and less often the axillary region. We present five different patients with this disease, thereby demonstrating its variation in clinical morphology. The lesion usually presents as an erythematous sharply defined spot. The polygonal borders, caused by the centrifugal growth of the tumor, may provide a diagnostic clue. The treatment of choice for extramammary Paget disease remains Mohs' microscopic surgery. However, radiotherapy or topical applications may be alternative treatment options in selected cases. In patients with the secondary form of extramam-mary Paget disease, treatment of the primary tumor is the main approach.