Abstract

We report a case of Extramammary Paget's disease occurring in a siblings. Case 1 was the elderly brother, age of 83-years old at 1994, suffered from an eczematous lesion on his genital region. Skin biopsy revealed that the lesion was Paget's disease. He had a complication of inguinal hernia, which was surgicaly removed at the time of operation of Paget's disease. Case 2 was the young brother of case 1, age of 73-years old at 1987, also had Paget's disease coexistent with inguinal hernia. Case 2 underwent an operation of Paget's disease, seven years before case 1. In our case, the pedigree suggests that Extramammary Paget's disease would have an autosomal ressesive inheritance. The pedigree also shows that the inguinal hernia which were seen in three of four generations had an autosomal dominant inheritance.