title: Xeroderma Pigmentosum - A case report with oral implications.
abstract: Xeroderma Pigmentosum is a rare autosomal recessive genetic disorder characterized by defective DNA repair leading to clinical and cellular hypersensitivity to ultraviolet radiation and carcinogenic agents. Important clinical features are: intense cutaneous photosensitivity, xerosis, poikiloderma, actinic keratosis, acute burning under minimal sun exposure, erythemas, hyperpigmented lentiginous macules, and malignant lesions in sun-exposed areas, including basocellular carcinoma, squamous cell carcinoma, and melanoma. There is a great involvement of many parts of the body, especially head and neck. The oral manifestations are mainly related to the occurrence of malignant tumors in the lips, tongue and buccal mucosa. This paper reports a rare case of Xeroderma Pigmentosum in a 41-year-old male presenting mainly dermatological, neurological and ophthalmological involvement. Oral implications such as severe oral pain and mouth opening limitation were present due to perioral scars. In addition, this paper discuss some important aspects concerning the role of the dental professional management of this entity, since XP patients require constant dental care and follow-up in order to control the occurrence of new lesions on the lips or inside oral cavity. Key words:Actinic cheilitis, oral involvement, Xeroderma pigmentosum.
authors.affiliation: DDS, MSc, PhD student, Department of Stomatology, Bauru School of Dentistry, University of São Paulo, Bauru, São Paulo, Brazil.
authors.forename: Camila
authors.initials: C
authors.lastname: Lopes-Cardoso
authors.affiliation: DDS, MSc, PhD student, Department of Stomatology, Bauru School of Dentistry, University of São Paulo, Bauru, São Paulo, Brazil.
authors.forename: Luciana M
authors.initials: LM
authors.lastname: Paes da Silva Ramos Fernandes
authors.affiliation: DDS, MSc. Department of Stomatology, Bauru School of Dentistry, University of São Paulo, Bauru, São Paulo, Brazil.
authors.forename: Julierme
authors.initials: J
authors.lastname: Ferreira-Rocha
authors.affiliation: MD, PhD. Lauro de Souza Lima Institute, Bauru, São Paulo, Brazil.
authors.forename: Cleverson
authors.initials: C
authors.lastname: Teixeira-Soares
authors.affiliation: MD, PhD. Lauro de Souza Lima Institute, Bauru, São Paulo, Brazil.
authors.forename: Jaison
authors.initials: J
authors.lastname: Antônio-Barreto
authors.affiliation: DDS, MSc, PhD. Department of Stomatology, Bauru School of Dentistry, University of São Paulo, Bauru, São Paulo, Brazil.
authors.forename: José
authors.initials: J
authors.lastname: Humberto-Damante