Case of Cowden syndrome associated with eccrine angiomatous hamartoma

Cowden syndrome, also known as multiple hamartoma syndrome is a rare autosomal dominant disorder characterized by multiple hamartomatous tumors of ectodermal, mesodermal and endodermal origin. A 47-year-old woman had a skin-colored plaque on the left foot. She had total abdominal hysterectomy due to uterine leiomyoma at the age of 35, and had modified radical mastectomy due to right breast cancer at 38. Thyroid adenoma was diagnosed at age 46. Physical examinations revealed macrocephaly, multiple papules of the buccal mucosa, skin tags of the neck and multiple keratotic papules of both hands. Multiple gastrointestinal polyps were detected on gastrofiberoscopy and colonoscopy. Histological examination of the skin lesion on the left foot showed an increased numbers of eccrine sweat glands and blood vessels, which are characteristic histological findings of eccrine angiomatous hamartoma (EAH), a rare benign tumor. We present a case of Cowden syndrome with an associated EAH, which has not been described in the English literatures.