Cowden syndrome

Cowden's disease (CD) is a rare syndrome (little more than 100 cases reported in the literature) [1], inherited as an autosomal dominant trait with an incomplete penetrance and variable expressivity [2] and characterized by multiple hamartomas and neoplasms of ectodermal, mesodermal and endodermal origin, predominantly localized to skin, breast, thyroid gland and gastrointestinal tract. On this subject we describe a case of a patient with oral papillomatosis, acral keratoses, intestinal polyps and goiter.