Perianal Paget's disease

Abstract

We report the case of a 74-year-old female suffering from an exudative erythematous lesion in the perianal region that after being treated topically several times, was diagnosed histologically of perianal Paget's disease. The presence of an invasive carcinoma was excluded. It was treated surgically with radical excision with wide resection margins and plastic reconstruction. Paget's disease affecting the perianal region is a rare condition of unknown ethiology. The extramammary location of this disease may be associated to apocrine swet gland carcinoma or to other primary carcinomas, such as rectum, prostate, bladder, etc. although occasionally the primary location cannot be determined. Its tendency to malignization is not great but, as it frequently associates to other malignancies, an early diagnosis is essential. Differential diagnosis may be difficult and it must be done against melanoma, Bowen's disease, leucoplasia, fungoid mycosis, squamous cell carcinoma, basaloid carcinoma, perianal psoriasis and condyloma acuminatus. It can only be confirmed by biopsy, the presence of Paget's cells being pathognomonic. Symptoms are inspecific, itching being the most common due. Diagnostic biopsies must be performed in any patient refering persistent anal itching. A radical and aggressive treatment is recommended due to its frequent association to other malignancies and its great tendency to local recurrence.