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2E67.11 Vulvar Paget disease

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

An uncommon intraepithelial malignant neoplasm of eccrine or apocrine origin, arising from the vulva. It usually affects post-menopausal women. In approximately 10-20% of the cases there is an associated anorectal, or urothelial carcinoma or a skin appendage adenocarcinoma identified. It presents as a red, eczematous lesion. Microscopically, it is characterised by the presence of the typical Paget cells which are large, round cells with abundant cytoplasm and prominent nuclei.

postcoordination

synonyms

• Vulvar Paget disease
• Extramammary Paget disease of vulva

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