Primary extramammary Paget disease (EMPD) is a rare malignant tumour occurring in apocrine gland-bearing areas, such as the genitals (mean age at diagnosis 60–70 years).1 Clinically, EMPD exhibits well-circumscribed, erythematous and scaly patches or plaques often mimicking inflammatory skin disorders, but occasional pigmentary changes such as hypo- or hyperpigmentation are reported.