Table 1.

Case reports of familial extramammary Paget's disease

Case reports	Patients	Location	Metastasis site	Underlying malignancy
Kuehn et al., 1973 [3]	1. Male 66 yr. (Father)	Scrotum	Right inguinal LN	No
	2. Male 58 yr. (Son)	Scrotum	No	No
Sakamoto et al., 1988 [12] 1. Male 57 yr. (Brother)	Scrotum	Right inguinal LN	NA
	2. Male 55 yr. (Brother)	Scrotum	No	NA
Amano et al., 1994 [13]	1. Male 59 yr. (Brother)	Scrotum	No	NA
	2. Female 56 yr. (Sister)	Labia majora	No	NA
Hashimoto et al., 1995	1. Male 83 yr. (Brother)	Scrotum, shaft of penis	NA	No
[14]	2. Male 72 yr. (Brother)	Scrotum, shaft of penis	NA	No
Nishibo et al.,	1. Male 81 yr. (Father)	Scrotum, shaft of penis	NA	NA
1996 [15]	2. Male 50 yr. (Son)	Scrotum	NA	NA
Mochizuki et al., 1998 [16] 1. Male 68 yr. (Father)	Scrotum	No	NA
	2. Male 47 yr. (Son)	Scrotum	No	NA
Demitsu et al., 1999 [17]	1. Male 74 yr. (Brother)	Mons pubis	No	No
	2. Female 66 yr. (Sister)	Mons pubis	No	No
Inoue et al., 2000 [8]	1. Female 83 yr. (Sister)	Vulvar	No	No
	2. Male 83 yr. (Brother)	Scrotum	No	Gastric adenocarcinoma
Zhang et al., 2015 [18]	1. Male 67 yr. (Brother)	Scrotum	Multiple lymph LN	No
	2. Male 56 yr. (Brother)	Scrotum	No	No
Rao et al., 2015 [19]	1.Male 56 yr. (Brother)	Scrotum	No	No
	2. Male 68 yr. (Brother)	Scrotum, root of penis	Left inguinal LN	No
Cases reported in this	1. Female 61 yr. (Sister)	Anogenital	No	No
Study	2. Female 67 yr. (Sister)	Anogenital	No	No

LN, lymph node; NA, not applicable; yr, year.