[Cowden's disease. A syndrome with multiple hamartomas and neoplasias]

We describe a patient with Cowden's disease, a rare genodermatosis characterized by multiple hamartomas and neoplasms of ectodermal, mesodermal and endodermal origin. The most characteristic findings are mucocutaneous lesions, including facial trichilemmomas, oral papillomatosis, and acral and palmoplantar keratoses. It is important to recognize these markers because of the high incidence of internal malignancy, the most common being breast cancer. Multiple extracutaneous hamartomas are described as being predominantly localized to breasts, thyroid gland, and gastrointestinal tract. Associated anomalies in multiple organs are frequently found.