EXTRAMAMMARY PAGET DISEASE

Course overview

EXTRAMAMMARY PAGET DISEASE (EMPD) IS A RARE DERMATOLOGIC CONDITION THAT FREQUENTLY PRESENTS IN AREAS WHERE APOCRINE SWEAT GLANDS ARE ABUNDANT. MOST COMMONLY, THIS INCLUDES THE VULVA, ALTHOUGH PERINEAL, SCROTAL, PERIANAL, AND PENILE SKIN MAY ALSO BE AFFECTED. LESIONS CLINICALLY PRESENT AS ERYTHEMATOUS, WELL-DEMARCATED PLAQUES THAT MAY BECOME EROSIVE, ULCERATED, SCALY, OR ECZEMATOUS. EXTRAMAMMARY PAGET DISEASE HAS A FEMALE PREDOMINANCE AND USUALLY OCCURS IN THE SIXTH TO EIGHTH DECADES OF LIFE. PROFESSIONALS DISAGREE ABOUT MANY ASPECTS OF EMPD, FOR EXAMPLE, THE PREVALENCE OF CONCURRENT VULVAR ADENOCARCINOMA OR INVASIVE EMPD, ASSOCIATION WITH REGIONAL AND DISTANT CANCERS, AND RECURRENCE RATES FOLLOWING SURGICAL EXCISION. EARLY RECOGNITION IS IMPERATIVE BECAUSE THE DIAGNOSIS IS FREQUENTLY DELAYED AND THERE IS A HIGH INCIDENCE OF ASSOCIATED INVASIVE DISEASE. THIS ACTIVITY DESCRIBES HOW TO PROPERLY EVALUATE FOR EXTRAMAMMARY PAGET DISEASE AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN CARING FOR PATIENTS WITH THIS CONDITION.