慢性皮肤黏膜念珠菌病的研究进展OA
Research progress on chronic mucocutaneous candidiasis
慢性皮肤黏膜念珠菌病(chronic mucocutaneous candidiasis,CMC)是一组以皮肤、甲、口腔和生殖器黏膜反复或持续发生念珠菌感染超过6个月为特征的疾病.本文对CMC的病因及发病机制、临床表现、诊断、治疗等方面进行综述,以期为相关临床诊疗工作提供参考.目前研究认为,CMC是一种免疫缺陷病,发病机制复杂.CMC患者在防御念珠菌感染的非特异性免疫和(或)特异性免疫中存在各种缺陷,导致患者无法抵御念珠菌感染.根据病因,CMC可分为原发性CMC和继发性CMC,原发性CMC多与基因突变导致的辅助性T细胞17(T helper cell 17,Th17)及白细胞介素-17(interleukin-17,IL-17)的免疫缺陷有关,而继发性CMC常与人类免疫缺陷病毒感染、糖尿病、接受免疫抑制治疗等因素相关.原发性CMC常表现为念珠菌感染,且不同基因突变导致的CMC常有不同伴发症状;继发性CMC除浅表器官的念珠菌感染和原发疾病的表现外,还可伴有深部真菌感染.对CMC进行诊断除需结合病史、临床特点外,还依靠涂片镜检、真菌培养、免疫学检查及基因测序和分析等辅助检查的结果,诊断为原发性CMC需排除上述继发性因素.目前三唑类、棘白菌素类、多烯类等抗真菌药为CMC的主要治疗方法,JAK抑制剂等生物制剂的免疫疗法为CMC患者的临床治疗提供了更多的选择,基因治疗亦有潜在的临床应用价值.
Chronic mucocutaneous candidiasis(CMC)is an infectious phenotype characterized by recurrent or per-sistent infections caused by Candida species that affect the skin,nails,oral,and genital mucosae for a duration exceed-ing six months.Current research suggests that CMC is an immunodeficiency disease with a complex pathogenesis.Pa-tients with CMC have various defects in nonspecific and/or specific immunity against Candida infection,resulting in the inability of patients to defend themselves against Candida infection.CMC can be stratified into primary CMC and sec-ondary CMC based on etiology.Primary CMC is often associated with genetic mutations leading to immunodeficiencies in T helper cell 17 and interleukin-17,whereas secondary CMC is frequently linked to factors such as human immuno-deficiency virus infection,diabetes mellitus,and immunosuppressive therapy.Primary CMC typically manifests as Can-dida infections,with distinct genetic mutations often correlating to varied concomitant symptoms.Secondary CMC may present with not only superficial mucosal Candida infections and manifestations of the underlying primary disease but also with invasive fungal infections.Diagnosing CMC requires an integration of medical history and clinical presenta-tion,supplemented by the outcomes of auxiliary diagnostic procedures,including microscopic examination of fungal smear,fungal culture,immunological testing,and genetic sequencing and analysis.Furthermore,confirming primary CMC requires exclusion of the aforementioned secondary factors.At present,antifungal drugs such as triazoles,echino-candins,and polyenes are the main treatment for CMC.Moreover,immunotherapy with biologics such as Janus kinase(JAK)inhibitors provides more options for the clinical treatment of patients with CMC.Gene therapy also has potential clinical application value.In this review,we discuss the etiologies,pathogenesis,clinical manifestations,diagnosis,and treatments of CMC,aiming to provide a reference for the clinical diagnosis and treatment of CMC.
饶晨星;梁静;莫龙会;王冏珂;曾昕
四川大学华西口腔医院口腔黏膜病科 口腔疾病防治全国重点实验室 国家口腔医学中心 口腔疾病国家临床医学研究中心 口腔黏膜病国家临床重点专科 中国医学科学院口腔黏膜癌变与防治创新单元,四川 成都(610041)成都市金牛区妇幼保健院口腔科,四川 成都(610081)四川大学华西口腔医院口腔黏膜病科 口腔疾病防治全国重点实验室 国家口腔医学中心 口腔疾病国家临床医学研究中心 口腔黏膜病国家临床重点专科 中国医学科学院口腔黏膜癌变与防治创新单元,四川 成都(610041)四川大学华西口腔医院口腔黏膜病科 口腔疾病防治全国重点实验室 国家口腔医学中心 口腔疾病国家临床医学研究中心 口腔黏膜病国家临床重点专科 中国医学科学院口腔黏膜癌变与防治创新单元,四川 成都(610041)四川大学华西口腔医院口腔黏膜病科 口腔疾病防治全国重点实验室 国家口腔医学中心 口腔疾病国家临床医学研究中心 口腔黏膜病国家临床重点专科 中国医学科学院口腔黏膜癌变与防治创新单元,四川 成都(610041)
医药卫生
慢性皮肤黏膜念珠菌病口腔念珠菌病念珠菌感染免疫缺陷基因突变辅助性T细胞17白细胞介素-17抗真菌药物免疫疗法
chronic mucocutaneous candidiasisoral candidiasisCandida infectionimmunodeficiencygene mutationT helper cell 17interleukin-17antifungal drugsimmunotherapy
《口腔疾病防治》 2026 (2)
191-201,11
国家自然科学基金项目(82470983,82270986) This study was supported by the grants from National Natural Science Foundation of China(No.82470983 and No.82270986).
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