Paget Disease

PAGET DISEASE

Paget disease is characterized by extensive intraepithelialdisease whose gross appearance is described as a fiery red background mottled with whitish hyperkeratotic areas. The histology of these lesions is similar to that of the breast lesions, with large, pale cells of apocrine origin below the surface epithelium (Fig. 42.4). Although not common, Paget disease of the vulva may be associated with carcinoma of the skin. Similarly, patients with Paget disease of the vulva have a higher incidence of underlying internal carcinoma, particularly of the colon and breast.

The treatment for vulvar Paget disease is wide local excision or simple vulvectomy, depending on the amount of involvement. Recurrences are more common with this disorder than with VIN, necessitating wider margins when local excision or vulvectomy is performed.