Perianal Paget's disease

An early case of perianal Paget's disease is described in a 59 year old female. Literature search revealed 57 documented cases up to 1987. Diagnosis is suspected on clinical features and needs to be always confirmed by biopsy with routine stains, and, in doubtful cases, also by electron microscopy and immunohistology. The extent of the primary disease and the presence of an associated adnexal or anorectal cancer must be established. Wide surgical excision of the primary lesion, preferably with frozen section examination of the excised margins and undersurface, is the preferred treatment. Associated adnexal or anorectal cancer is treated on merit. Recurrent disease is managed by further excision, topical chemotherapy and/or laser therapy. Prognosis depends on the extent of the primary lesion, on the adequacy of the primary excision, and on the presence of an associated adnexal or anorectal cancer. In apparently cured cases, long-term surveillance is recommended.