Incisional biopsy
Extramammary Paget’s
disease may be found in the axilla and in the anogenital region, including the
labia majora, penis, scrotum, groin, pubic area, perineum, perianal region,
thigh, and buttock. Paget’s disease of the perianal area is a malignant neoplasm
of the intraepidermal portion of apocrine glands with or without associated
dermal involvement. Paget’s disease has a long preinvasive phase, but if
untreated, an invasive adenocarcinoma of the apocrine gland type develops. The
disease is more common in women than men, with the highest incidence in the
seventh decade.
Macroscopically, the lesion appears as an erythematous scaly or eczematoid
plaque-like lesion, similar to other benign perianal lesions, making clinical
diagnosis difficult. A definite diagnosis is made by biopsy, which shows
characteristic histologic appearance—large, pale, vacuolated cells with
hyperchromatic eccentric nuclei. The cells invariably contain acid
mucosubstances, an important feature in distinguishing this lesion from melanoma
and Bowen’s disease
