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慢性皮肤粘膜念珠菌病是一种少见的慢性进行性念珠菌感染,临床表现为一组综合征。病人先后出现皮肤、指甲、粘膜的反复性念珠菌感染,多伴有免疫或内分泌的失调,致病菌主要为发/毛癣菌属和小孢子菌属。目前有关的免疫学研究显示该病主要为T淋巴细胞功能缺陷所致。至今为止,唯一被证实与该病明确相关的基因为自身免疫调节因子,该基因为自身免疫多内分泌腺病—念珠菌病—外胚层发育不全综合征)的致病基因。本文主要对慢性皮肤粘膜念珠菌病的分类、临床表现,免疫病理及基因研究进行综述。
Chronic mucocutaneous candidiasis is a rare chronic progressive Candida infection, a clinical manifestation of a syndrome. Patients have repeatedly skin, nails, mucosal recurrent Candida infections, often accompanied by immune or endocrine disorders, pathogens mainly hair / Trichophyton and Microsporum genus. The current immunological studies have shown that the disease is mainly due to T lymphocyte dysfunction. To date, the only gene that has been shown to be clearly associated with the disease is an autoimmune regulatory factor that is the causative gene of autoimmune polyendocrinopathies - candidiasis - ectodermal syndrome. This article mainly reviews the classification, clinical manifestation, immunopathology and gene of chronic mucocutaneous candidiasis.