容易漏诊的一种大疱病——p200类天疱疮  

Anti-p200 Pemphigus:An Easily Misdiagnosed Bullous Disease

作　　者：左亚刚[1] ZUO Yagang(Department of Dermatology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College)

机构地区：[1]中国医学科学院北京协和医院皮肤科,北京100730

出　　处：《皮肤科学通报》2021年第3期253-258,共6页Dermatology Bulletin

摘　　要：p200类天疱疮是一类罕见的自身免疫性大疱病,其临床表现不特异,易与大疱性类天疱疮、获得性大疱性表皮松解症等其他大疱病混淆。研究发现大多数p200类天疱疮患者的血清可识别层粘连蛋白γ1,故p200类天疱疮又称为抗层粘连蛋白γ1型类天疱疮。该病多发生于中老年男性,临床表现为躯干和四肢紧张性水疱或大疱,部分表现为荨麻疹样斑块。瘢痕和粟丘疹少见。病理表现为表皮下水疱,真皮乳头中性粒细胞浸润为主要特点,甚至在真皮乳头出现中性粒细胞微脓疡。皮损周围正常皮肤的直接免疫荧光显示在真皮-表皮连接处线状IgG沉积,盐裂间接免疫荧光证实致病抗体结合于皮肤真皮侧(水疱底),且免疫印迹法检测自身抗体可与人真皮提取物中200 kDa蛋白(p200)结合。其治疗方法与大疱性类天疱疮类似,糖皮质激素和氨苯砜有效。Anti-p200 pemphigoid is a kind of rare autoimmune bullous disease.Its clinical manifestations are not specific,and it is easy to be confused with bullous pemphigoid,acquired epidermolysis bullosa and other bullous diseases.It is reported that the serum of most patients with P200 pemphigoid can recognize laminin γ1,so P200 pemphigus is also called anti-laminin γ1 pemphigoid.The disease mostly occurs in middle-aged and elderly men.The clinical manifestations are tension blisters or bullae on the trunk and limbs.Urticaria-like plaques can also be found in some patients.Scars and milia are rare.The main pathological features include epidermis blister formation,infiltration of neutrophils in dermal papilla,and even micro abscess of neutrophils in dermal papilla.Direct immunofluorescence of a perilesional skin biopsy showed linear IgG deposition at the dermal epidermal junction.The pathogenic antibody can bind to the dermal side of the skin(blister floor)by indirect immunofluorescence on salt-split skin,and the autoantibody can react with a 200 kDa protein(p200)in extract of human dermis by immunoblotting.Similar to bullous pemphigoid,glucocorticoid and dapsone are effective.

关 键 词：p200类天疱疮 抗层粘连蛋白γ1 大疱性类天疱疮 获得性大疱性表皮松解症 诊断 治疗 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]