[Paget's extramammary disease of the axillae and perineum]

Paget's extramammary disease mostly affects genital, perianal and axillary regions. Whilst triple involvement has been described in Japanese patients, simultaneous lesions of both axillary regions and the inguinal area are exceptional among European patients. We report a case of triple Paget's extramammary disease in a Caucasian patient.

A 79-year-old male patient who developed a prostatic adenocarcinoma 3 years ago, was seen for an erythemato-squamous intertrigo of both axillary folds and the pubic area, present for 10 years, not diagnosed and resistant to topical treatments. Triple Paget's extramammary disease was confirmed by both histopathological and immunohistochemical investigations. No recurrence of the prostatic adenocarcinoma was observed.

Since the first description of triple Paget's extramammary disease, 28 cases have been reported in Japan. To our knowledge, this is the first case observed in a Caucasian patient. The clinical features of axillary lesions are described as pigmented or depigmented plaques, sometimes lichenoid or erosive. For some Japanese authors, a biopsy is mandatory even in the absence of clinical lesions, since typical Paget cells can be found. Immunohistochemical studies reveal CK7 expression, the marker of choice for primary extramammary Paget's disease. CK7 - would suggest underlying regional internal malignancy as well as CK20 +. Despite the fact that the immunophenotype was CK7 +/CK20- the patient developed an evolving prostatic adenocarcinoma. Although various treatments are described in the literature, surgical excision remains the first line treatment whenever possible.