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帕皮永-勒费弗尔综合征:口腔表现与治疗

Papillon-Lefèvre syndrome: Oral aspects and treatment.

作者信息

Giannetti Luca, Apponi Roberto, Dello Diago Alberto M, Jafferany Mohammad, Goldust Mohamad, Sadoughifar Roxanna

机构信息

Department of Surgery, Medicine, Dentistry and Morphological Sciences with Transplant Surgery, Oncology and Regenerative Medicine Relevance, University of Modena and Reggio Emilia, Modena, Italy.

Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.

出版信息

Dermatol Ther. 2020 May;33(3):e13336. doi: 10.1111/dth.13336. Epub 2020 Apr 9.

Abstract

Papillon-Lefèvre syndrome (PLS) is a rare disorder characterized by diffuse palmoplantar erythematous, fissured hyperkeratosis, and aggressive periodontal disease that starts in the early periods of childhood. Periodontal disease occurs with the early loss of deciduous teeth at the age of 2 to 4 years, followed by the loss of permanent teeth during adolescence. Prosthodontics management of PLS patients is very complex and sometimes requires invasive therapeutic treatments. Early diagnosis is essential for correct treatment management avoiding the possibility that patients are early edentulous. Management could be a conventional periodontal treatment and pharmacological therapy but in severe cases, digital techniques, could be help the clinician for increased patient comfort and minimized tissue damage.

摘要

掌跖角化-牙周破坏综合征(PLS)是一种罕见的疾病,其特征为弥漫性掌跖红斑、皲裂性角化过度以及始于儿童早期的侵袭性牙周病。牙周病在2至4岁乳牙早期脱落时出现,随后在青春期恒牙脱落。PLS患者的口腔修复治疗管理非常复杂,有时需要侵入性治疗。早期诊断对于正确的治疗管理至关重要,可避免患者过早无牙。治疗可以是传统的牙周治疗和药物治疗,但在严重病例中,数字技术有助于临床医生提高患者舒适度并将组织损伤降至最低。

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