Triple extramammary paget's disease

BACKGROUND. Triple extramammary Paget's disease (TEPD) has been considered to be rare in the English literature, and its incidence and characteristics are unclear. There are many therapeutic options for treating extramammary Paget's disease (EPD). OBJECTIVE. Our purpose was to investigate how many TEPD cases have been reported previously and to describe their characteristics. We also describe the effectiveness of radiotherapy for them. METHODS. We report two TEPD cases, and summarize previously reported TEPD cases together with our cases. RESULTS. Twenty-three TEPD cases have been reported previously. Of these, 19 cases have been in Japan. All but one patient with TEPD were male. Their axillary lesions often showed no eruptions or very slight erythema. Radiotherapy for our cases was effective although the effectiveness of radiotherapy is controversial. CONCLUSION. In genital Paget's disease bilateral axillae should be examined histologically, even if they show no or slight eruptions. Radiotherapy may be useful for EPD, particularly axillary Paget's disease.