Abstract
Four cases of extramammary Paget's disease (EMPD) were reported to have siblings with the same disease. Three cases were brothers, one case was a father and his son. Thus, 15 cases of familial EMPD were reported in the literature mainly from Japan. Evaluation of these case reports revealed (1) familial occurrence of EMPD may not be so rare but may be ignored, so a detailed and prospective family history should be taken ; (2) in the cases of parents and children, the children were diagnosed to have EMPD at a much younger age. This could be explained by their higher interest in the disease, and may suggest that true age of onset of EMPD is much younger ; (3) the involved regions were similar in each familial member, which suggest the genetic base of MPD.[Skin Cancer (Japan) 2013 ; 28 : 174-180]
