Perianal Paget disease: Case report

Reporte de Caso

Abstract

Perianal Paget disease (PPD) was reported for the first time in 1893 and was included as a subgroup within extramammary Paget disease (EMPD). Between the last ones, which most frequent location is the vulvar, PPD represent less than 1% of all the anal diseases and 6,5% of all the cases of EMPD. Mammary Paget disease and EMPD, share the presence of Paget cells in the epidermis and a similar clinical presentation, but the difference lays in the anatomic localization and the histogenesis. Due to the low frequency of the disease, there are not big studies to investigate the optimal treatment, which remains controversial. We present a clinical case of a 63-years-old female patient with a 4-years-old history of a pruritic lesion in the perianal region. Physical examination revealed an erythematous lesion of 5 cm diameter, that compromised both posterior quadrants and the midline. Rectal digital examination was normal and the was not presence of inguinal adenopathy. A biopsy was performed and the diagnosis of PPD was made. The decision of surgical treatment was made, which consisted in local excision with wide margins and primary closure. The pathology report informed EMPD with intraepidermal lesion and margins free of disease. The patient had a full recovery and after a follow up of two years, there is not evidence of local recurrence.