家族性良性慢天疱疮的非常态.pdfVIP

家族性良性慢性天疱疮的非常态 中文摘要 家族性良性慢性天疱疮的非常态 中文摘要 家族性慢性良性天疱疮（Familial Benign Chronic Pemphigus，FBCP ）是一种少见 的常染色体显性遗传性大疱性皮肤病，临床上以疼痛性糜烂、水疱及脱屑性红斑块为 特征，主要发生于摩擦部位如颈部、腋下、腹股沟及会阴部。近几年在临床上逐渐可 见到不典型的病例，包括少见部位及不典型皮损损害如斑丘疹、角化性丘疹、乳头瘤 样增殖病变、掌趾部偶有点状小坑及散在分布的角化也偶可见到。本文就近年来文献 资料中有关该病的不典型临床表现包括发病年龄、基因、诱发因素、皮疹情况、组织 病理学、合并情况、治疗反应等方面做一综述。 关键字：家族性慢性良性天疱疮；Hailey-Hailey 作者：杨晓雯 指导老师：施辛 I 英文摘要 家族性良性慢性天疱疮的非常态 Atypical Manifestation of Hailey-Hailey Disease Abstract Hailey-Hailey disease (familial benign chronic pemphigus) is a rare intraepidermal blistering disorder, which is inherited as an autosomal dominant trait. It is clinically characterized by recurrent vesicles which rupture, leaving painful fissures (rhagades) and scaly erythematous plaques on the neck, axilla, groin, and in intertriginous areas. In recent years, the atypical cases can be observed in the clinic gradually, including rare and atypical lesions such as maculopapule, keratosis papules, papillomatosis, discrete punctate palmar keratoses. The aims of this paper were to offers an update on the knowledge about the age of onset,genemutation,disease modifying factors,pattern of disease and clinical manifestations, histopathoiogy, complications and treatment of Hailey-Hailey disease. Key words ：Familial benign chronic pemphigus;Hailey-Hailey disease Written by: Yang Xiao Wen Supervised by: Prof.Xin Shi