Extramammary Paget’s disease

A 72-year-old man presented with a solitary eczematous perianal lesion causing a painful pruritus. A biopsy showed an in situ adenocarcinoma with typical Paget’s cells. Treatment consisted of a radical excision. Extramammary Paget’s disease is a rare cutaneous cancer involving skin areas rich in apocrine sweat glands, such as in the genital region, perineum or axilla. Unlike mammary Paget’s disease, there is a high risk (approximately 30 per cent) of other malignant tumours.