Cowden's syndrome: a case report.

ABSTRACT

Cowden's syndrome, a rare genodermatosis of autosomal-dominant inheritance with variable expressivity, is characterized by a combination of ectodermal, mesodermal, and endodermal hamartomas that may involve the skin, mucous membranes, breasts, gastrointestinal tract, and thyroid. A 26-year-old woman who presented for replacement of her teeth, all of which had been extracted because of rapidly progressive periodontitis. She was diagnosed with Cowden's syndrome based on mucocutaneous abnormalities, thyroid involvement, and abnormalities of the skeletal and genitourinary systems. The clinical significance and differential diagnosis of this disease are highlighted.