Xeroderma pigmentoso: manifestações orais, implicações odontológicas e reabilitação bucomaxilofacial: revisão de literatura e relatos de caso

Abstract

Xeroderma pigmentosum (XP) is a rare genetic disease characterized by defects in DNA repair mechanisms, resulting in high sensitivity to ultraviolet radiation and a predisposition to the early development of neoplasms in photoexposed areas, such as skin, lips, eyes, and oral mucosa. Tumors such as basal cell carcinoma and squamous cell carcinoma are frequent, with surgical removal being the main form of treatment, which can result in extensive facial defects. In this context, the present study aimed to analyze the oral manifestations, dental management, and rehabilitative approaches in patients with XP, through a literature review, associated with the presentation of two clinical cases. As this was a literature review, the search was conducted in the PubMed, BVS, and Embase databases, considering studies published in the last 15 years, in English and Portuguese, including clinical studies, reviews, and case reports. The results showed a high frequency of malignant neoplasms, especially squamous cell carcinoma in the lips and tongue, in addition to oral alterations and the need for continuous dental follow-up. The clinical cases included patients with extensive facial losses, rehabilitated with adhesive-retained silicone prostheses, demonstrating challenges in the molding, sculpting, aesthetic characterization, and prosthesis retention stages. It is also observed that there is a limitation in the available literature, with a predominance of case reports and a scarcity of systematized studies on the subject. It is concluded that maxillofacial rehabilitation plays a fundamental role in the aesthetic, functional, and psychosocial restoration of these patients, and an interdisciplinary and individualized approach is essential.