Abstract

Cowden's syndrome (CS) is a complex and rare disorder of autosomal dominant inheritance with a variable phenotype. It is characterized by multiple malignant and benign hamartomatous lesions affecting several tissues and organs derived from the three germ layers. The organ system that most consistently manifests this syndrome is the skin. Mucocutaneous lesions are present in 99-100% of cases. The importance of this disease lies in the increased susceptibility to malignization of some lesions, especially breast, thyroid, and genito-urinary tract lesions. As a result, the disease has been considered a preneoplastic condition. Different types of management regimes have been advised for such patients (management of breast cancer, management of bone metastasis). But in this case report, we present for the first time the complete oral rehabilitation of a patient with CS, which, according to our knowledge, is the first documented case of its kind.

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