Very rare malignancy originating in vulvar apocrine-gland-bearing skin cells (primary disease) or as a manifestation of adjacent primary anal, rectal or bladder adenocarcinoma (secondary disease)

Aetiology

• Usually only affects post-menopausal women

Pathophysiology

• Tumour cells in epidermis, contain mucin

Clinical presentation

• Crusting rash, often sharp demarcation
• Pruritic/painful

Investigations

• Biopsy
• Further imaging may be used to investigate for further malignancy

Management

• Options include surgery, photodynamic therapy, topical imiquimod, and radiotherapy