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
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