ICD-11 classes
02 Neoplasms
In situ neoplasms, except of lymphoid, haematopoietic,...
2E64 — Carcinoma in situ of skin
2E64.1 — Extramammary Paget disease of skin
2E67.11 — Vulvar Paget disease

ICD-11 2E67.11 — Vulvar Paget disease

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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