1、大疱性皮肤病大疱性皮肤病 概概 述述Introduction大疱性皮肤病是指一组发生在皮肤黏膜以大疱为基本损害的皮肤病。病因包括自身免疫和遗传等，前者包括天疱疮、大疱性类天疱疮，后者包括家族性慢性良性天疱疮和大疱性表皮松解症。天天 疱疱 疮疮PEMPHIGUS天疱疮是一组累及皮肤和粘膜的自身免疫性表皮内大疱病。共同的特征有：薄壁、松弛易破的大疱；组织病理为表皮内水疱；免疫病理显示角质细胞间IgG、IgA、IgM 或C3的网状沉积。Pemphigus.ruptured bullae with crustPemphigus.HistopathologyPemphigus.Acantholysisi
2、mmunofluorescence(IF)IgG IgA IgM&C3 netty deposition at interkeratinocyte病因及发病机理病因及发病机理患者血中有抗角质形成细胞间物质抗体，其与角质形成细胞间物质结合，使细胞释放纤维蛋白酶原激活物，导致细胞间粘合物质降解，引起表皮棘层细胞松解。Circulating intercellular antibodies(pemphigus antibody 天疱疮抗体)are present in the patients serum,and antibody titers often parallel disease acti
3、vity.角质形成细胞间物质？桥粒desmosomes临床分型临床分型Classification 寻常性天疱疮pemphigus vulgaris 增殖性天疱疮pemphigus vegetans 落叶性天疱疮pemphigus foliaceous 红斑性天疱疮pemphigus erythematous寻常性天疱疮寻常性天疱疮 pemphigus vulgaris 口腔黏膜常受累 好发于头颈、胸背部 松弛性大疱、壁薄、易破 尼氏征阳性 The Nikolsky sign is positive pemphigus vulgaris occurs with equal frequency
4、in men and women,usually their fifth and sixth decades.It is rare in young persons.The mouth lesions appear first in 60%of cases.generalized bullous phase in most patients occurs some 5 or more months after the onset of oral lesions.增殖性天疱疮增殖性天疱疮pemphigus vegetans 好发于腋窝、乳房下、腹股沟、外阴、肛周等部位创面上有肉芽增殖尼氏征阳性有
5、臭味 pemphigus vegetans is characterized by flaccid bullae that bacome erosions and form fungoid(蕈状的)vegetations(增殖)or papillomatous(乳头瘤样)proliferations(增生),especially in body foids(皱褶),and reducing the foul(恶臭)odor(气味).落叶性天疱疮落叶性天疱疮pemphigus foliaceous好发于中老年人；口腔黏膜受累少见；疱壁极薄、易破，形成黄褐色油腻性叶片性痂和鳞屑；尼氏征阳性。pem
6、phigus foliaceus is a relatively mild,chronic variety of pemphigus characterized by flaccid bullae and localized or generalized exfoliation(剥脱).PF begins with small,relatively flaccid bullae,the bullae soon rupture to form erosion which covered with filemot(黄褐色)greasy(油腻性)crusts.红斑性天疱疮红斑性天疱疮pemphigu
7、s erythematous好发于头面部、胸背上部红斑、水疱尼氏征阳性黏膜损害少见 The leision are erythematous and thickly crusted,bullous or even hyperkeratotic(角化过度).These are usually localized on the nose,cheeks,and earsthe sites frequently affected by lupus erythematosis mucous membranes lesion are rarely seen.诊断依据诊断依据 Diagnosistypica
8、l clinical features 松弛性水疱、易破、不易愈合；尼氏征阳性 可伴有口腔黏膜受损 组织病理为表皮内水疱；免疫病理显示角质形成细胞间IgG、IgA、IgM或C3的沉积。Biopsy material(活检)Because the bullae of pemphigus become large and flaccid in a short time.it is important that a small,early,intact blister be secured.治治 疗疗Treatment 一般治疗General treatment：营养支持疗法 糖皮质激素：早期使用、
9、足量控制、合理减量、长期维持。免疫抑制剂：常与激素联合用药 血浆置换疗法 抗感染疗法 局部用药TreatmentTreatment Systemic therapy Corticosteroid therapy is the standard treatment of choice.the sooner the diagnosis is established and the sooner treatment is given,the more favorable the prognosis.the therapeutic effects are estimated by the number
10、 of new vesicles per day and the rate of healing of the new lesion,Nikolsky sign,pemphigus antibody titers.medication is continued until clinical disease is suppressed and pemphigus antibody disappears from the serum.TreatmentTreatment Systemic therapySystemic therapy Corticosteroid therapyCorticost
11、eroid therapy prednisone area dosage 10%3040mg/d 3050%6080mg/d 50%80100mg/d or more原则原则：早期使用、足量控制、合理减量、长期维持早期使用、足量控制、合理减量、长期维持notice The clinician should remember that today the risk of death in pemphigus from the side effects of oral prednisone is greater than the risk of death from the disease its
12、elf.大疱性类天疱疮大疱性类天疱疮 bullous pemphigoid是一种自身免疫性表皮下大疱病。表现为水疱壁厚、不易破，组织病理为表皮下水疱，免疫病理示基底膜带IgG和C3线状沉积发病机理发病机理患者血清中有抗基底膜带的自身抗体；靶抗原为半桥粒上的BP230和BP180.IgG&C3 linear deposition on basement membrane zone(BMZ).Circulating BMZ antibodies of IgG class are present in the patients serum.No close correlation exists be
13、tween the titer of antibodies and clinical disease activity.临床表现临床表现clinical features 好发于50岁以上的中老年人 好发部位：躯干、四肢伸侧、腋窝、腹股沟 紧张性水疱Thick-walled,large,tense,subeppidermal bullae 尼氏征阴性The Nikolsky sign is negativeBullous pemphigoid.Bullous pemphigoid.ImmunopathologyIgG&C3 linear deposition on basement membr
14、ane zone诊诊 断断Diagnosis紧张性水疱、壁厚不易破组织病理为表皮下水疱免疫病理示基底膜带IgG和C3线状沉积治治 疗疗Treatment 糖皮质激素 Corticosteroid therapy is the standard treatment of choice.prednisone 0.51.0mg/(kg.d)免疫抑制剂 氨苯砜Dapsone 50300mg/d po 支持及抗感染 外用药物 课后提问：1.请简述桥粒的显微结构？2.请简述天疱疮与类天疱疮的鉴别要点？ReferenceRichard B.ODOM.Andrews Disease of the Skin(Tenth edition).科学出版社.2010张学军主编.皮肤性病学教材(第七版).人民卫生出版社.赵辨主编.中国临床皮肤病学(第四版).江苏科学技术出版社.2010期刊及网络资料
