Background: The mouth is the mirror of the body and can manifest signs of underlying health issues. The oral soft and hard tissues are physically linked to the rest of the body. Therefore, the role of dentists is much greater than the dental setup. Many physicians consider dentistry as a purely practical occupation and not scientific. In recent years, the relationship between oral and systemic disease has become a leading research issue. Interventional studies focused on the relationship between dental treatment and its effect on certain systemic condition. In this regard, the aim of this study is to introduce a case of Cowden Syndrome (CS) which was first diagnosed according to their oral manifestations by a dentist. Case report: A 37-year-old woman was referred to the Department of Oral Medicine, Shahid Beheshti University of Medical Sciences complaining of aesthetic problems. Intra-oral examinations showed grooves and papillomatosis. Examination of the oral cavity showed a high- arched palate, hypoplasia of the maxilla and jaw. According to the patient’s oral presentation, we asked for a consultation with gastroenterologist, gynecologist and endocrinologist. Finally, based on clinical and paraclinical findings, the clinicians put the final diagnosis of CS. Discussion: According to the review of literature, less than 500 cases have been reported worldwide, many of which have been diagnosed by dental surgeons, or oral pathology and medicine specialists by recognition of mucocutaneous lesions. There is a close association of CS with the development of several malignant neoplasms. This point emphasizes the importance of the early recognition of these lesions, which facilitates an early diagnosis of CS. Oral lesions can be the first sign of the disease, because they develop in 90 to 99% of cases. The diagnosis of CS in this case was made based on criteria proposed by the International Cowden Syndrome Consortium. Conclusion: Since the patients can have a variety of clinical manifestations, the diagnosis can be mistaken and delayed. Therefore, it seems that there is a need for awareness of this entity in the dental community.
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