Dental treatment for patients with Nakajo-Nishimura syndrome: Report of three cases

Publication date: Available online 9 January 2020Source: Journal of Oral and Maxillofacial Surgery, Medicine, and PathologyAuthor(s): Kenjiro Okamoto, Itaru Tojyo, Yukari Shintani, Takashi Nakanishi, Naoki Mizobata, Yuya Takeda, Shigeru Suzuki, Shinsuke Ieda, Masamichi Ueda, Nobuo Kanazawa, Shigeyuki FujitaAbstractNakajo-Nishimura syndrome, which is a specific autoimmune disorder characterized by chronic inflammation, emaciation, and exhaustion, is inherited in an autosomal recessive fashion and is representative of proteasome dysfunction. Its main clinical features include pernio-like red-to-purple skin plaques on the hands and feet, periodic fever, progressive localized atrophy of fat and muscle, emaciation, elongated and thickened fingers and toes, joint contractures, enlarged liver, basal ganglia calcification, and a heliotrope rash. The dental treatment for three patients with Nakajo-Nishimura syndrome is described. Dental extractions and procedures were successfully performed without causing any obvious intraoperative or postoperative complications, although all three patients showed progressive trismus. When performing a dental procedure in patients with proteasome dysfunction, preoperative antibiotic therapy should be given because they are susceptible to infection due to the long-term use of high-dose steroids. Drug-related osteonecrosis of the jaw should also be considered. Proteasome dysfunction is characterized by joint contractures and progressive fat and muscle ...

Source: Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology - January 10, 2020 Category: ENT & OMF Source Type: research