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Perianal (extramammary) Paget's disease is rare and corresponds to an intraepithelial adenocarcinoma arising from dermal apocrine sweat glands. Biopsy reveals the diagnosis. The condition is often associated with an underlying malignancy (carcinoma of the apocrine or eccrine glands, rectal carcinoma, anal carcinoma). Wide local excision is recommended when invasive growth is absent. For invasive cancer or when associated with a synchronous malignancy abdomino-perineal resection is the treatment of choice. Two cases of perianal Paget's disease are presented.
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