1994 Volume 36 Issue 6 Pages 1237-1243
A thirty-nine year-old Japanese man with Cowden's disease is presented. He had facial papules, aacal keratosis, gingival papillomatosis, and multiple follicular cyst on his breast and abdomen. He also had adenomatous goiter, multiple gastrointestinal polyps and pelvic arteriovenous malformations. A diagnosis of Cowden's disease with arteriovenous malformations is rare, he is the first case of Cowden's disease in Japan complicated with pelvic arterioven ous malformations. The dysfunction of the immune systems has been pointed out in Cowden's disease. Our H LA typing research revealed that, five of four patients had A2 antigen.