Bielecka-Kowalska Natalia P, Lewkowicz Natalia
Department of Oral Mucosal and Periodontal Diseases, Medical University of Lodz, Lodz, Poland.
Postepy Dermatol Alergol. 2023 Feb;40(1):8-15. doi: 10.5114/ada.2022.124678. Epub 2023 Feb 27.
Behçet's disease (BD) is a rare, chronic multisystem disease of unknown aetiology. Clinically, it presents with ulceration located on the oral and genital mucosa, skin, uveitis, arthritis and neurological disorders. Most commonly, the disease affects men in the second and third decades of life and occurs endemically. The vast majority of cases have been reported in Asia and the Mediterranean area. The diagnosis of this disease is difficult, especially because of its rare occurrence in Central Europe. In this report, we present the case of BD in a 56-year-old man with primary sclerosing cholangitis, followed by a literature review covering current insights into BD aetiology, diagnostics and treatment.
白塞病(BD)是一种病因不明的罕见慢性多系统疾病。临床上,其表现为口腔和生殖器黏膜溃疡、皮肤病变、葡萄膜炎、关节炎及神经系统疾病。该病最常累及二三十岁的男性,呈地方流行性。绝大多数病例报告于亚洲和地中海地区。由于在中欧罕见,该病的诊断较为困难。在本报告中,我们呈现了一例患有原发性硬化性胆管炎的56岁男性白塞病病例,随后进行文献综述,涵盖对白塞病病因、诊断及治疗的当前见解。