Extramammary Paget disease

Orpha code: 2800OMIM code: 167300

Definition

A rare skin tumor characterized by predominantly intraepithelial growth of an adenocarcinoma which may either arise primarily in the skin (primary extramammary Paget disease) or result from intraepithelial spread of a visceral carcinoma (secondary extramammary Paget disease). The lesion is typically located in the anogenital region, presenting as a scaly, oozing, pruritic or painful erythematous plaque often resembling eczema. It may exhibit an invasive component with a significant risk of lymph node metastasis.

Disease data
Classification

Disease

ORPHA code
2800
OMIM code
167300
ICD10 code
C44.5
ICD11 code
2E64.1

No additional description.

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