口腔膜大疱类疾病文档资料.ppt
总论,选择相应内容点击,BACK,天疱疮,瘢痕性类天疱疮,大疱性类天疱疮,其他大疱性疾病,本课小结,单纯疱疹Herpes simplex,NEXT,带状疱疹Herpes zoster,水痘Chicken pox,NEXT,多形红斑Erythema multiform,药物过敏性口炎Allergic medicamentosus stomatitis,NEXT,病毒感染性变态反应性自身免疫性创伤性斑纹类,NEXT,疱性损害,一.总论,BACK,疱的概念,疾病范畴,疱 vesicle 粘膜或皮肤内储存液体而成 水疱 water blister 疱内容物为浆液 血疱 blood blister 疱内容物为血液 脓疱 pustule 疱内容物为脓液大疱 bulla 直径 5mm,上皮内疱 上皮下疱,NEXT,血疱Blood blister,水疱Water blister,NEXT,BACK,上皮下疱subepithelial bulla,上皮内疱intraepithelial bulla,二.天疱疮（pemphigus）,1.概述(preface),严重(severity)致死性疾病 激素(corticosteroid)的使用降低了死亡率,NEXT,NEXT,慢性(chronicity)不易愈合 反复发作 皮肤 粘膜联合疾病(skin-mucosa associated disease)寻常型口腔损害多见 其他型口腔损害少见,分类classification,寻常型 pemphigus vulgaris增殖型 pemphigus vegetans叶 型 pemphigus foliaceus红斑型 pemphigus erythematous,NEXT,2.病因(pathogeny)不明 自身免疫性疾病 autoimmune disease 抗棘细胞层间粘合物质的自身抗体3.临床表现(clinical manifestation),NEXT,NEXT,4.病理(pathology)棘层松解 上皮内疱 棘层松解细胞,NEXT,5.诊断(diagnose)1）病史2）临床表现 寻常型：口腔损害最早 最重 增殖型：疱底肉芽组织增殖 叶型：皮损为主 松弛大疱 鳞屑痂边翘如叶 红斑型：皮损为主 面部对称红斑 鳞屑痂,NEXT,3）特殊检查活检细胞学：涂片寻找棘层松解细胞（天疱疮细胞）免疫荧光法：直接法 间接法 显示棘细胞层间抗细胞粘合物质的抗体4）全身情况：恶病质,NEXT,6.鉴别诊断(differential diagnoses),NEXT,7.治疗(treatment),支持疗法：高蛋白 高维生素饮食 水电解质平衡 输血皮质激素 首选 规则用药：起始 控制 减量 维持 加用免疫抑制剂可减少激素用量 加用抗生素预防继发感染 注意禁忌证和不良反应,NEXT,局部：0.5达克罗宁止痛 皮质散消炎中医中药,NEXT,增殖性天疱疮治疗前后,BACK,三.瘢痕性类天疱疮（cicatricial pemphigoid),概述 良性粘膜类天疱疮 benign mucosa membrane pemphigoid病因 自身免疫性疾病 抗基底膜抗体,NEXT,临床表现病理：上皮下疱 无棘层松解免疫荧光直接法 基底膜区连续细长荧光带,NEXT,5.诊断 临床表现 活检 免疫荧光法6.鉴别诊断 寻常型天疱疮 多形渗出性红斑 糜烂性扁平苔癣,NEXT,7.治疗,局部：抗炎 抗粘连全身：激素 红霉素,BACK,四.大疱性类天疱疮（bullous pemphigoid）,1.概述 病程长，预后好2.病因 自身免疫性疾病 抗基底膜抗体,NEXT,3.临床表现 口腔：少见 疱小 不易破 尼氏征()颊部多见 皮肤：外观正常皮肤或红斑的基础上起 大疱 尼氏征(),NEXT,大疱性类天疱疮,NEXT,大疱性类天疱疮,NEXT,4.病理 上皮下疱 无棘层松解 连续细长荧光带（直接免疫荧光）5.诊断：皮肤大疱 免疫荧光,NEXT,6.鉴别诊断,NEXT,7.治疗,激素氨苯砜 四环素 烟酰胺联合,BACK,五.其他大疱性疾病,（一）类天疱疮样扁平苔藓（lichen planus pemphigoides，LPP）同时有典型的LP、BP特征 又有独特的抗原结构分子,NEXT,1.临床表现 口腔：紧张性大疱网状白条纹 皮肤：与大疱性类天疱疮相似 尼氏征(),NEXT,2.诊断与鉴别 无公认诊断标准 有典型LP BP特征。基底膜带有IgG、C3线状沉淀，IIF血清中与抗基底膜带抗原结合沉淀 鉴别：大疱性扁平苔藓 大疱性类天疱疮 大疱性红斑狼疮 大疱性表皮松解症,NEXT,3.治疗,强的松硫唑嘌呤灰黄霉素,NEXT,（二）副肿瘤性天疱疮(paraneoplastic pemphigus,PNP)体内肿瘤的循环体液因子或其代谢产物的作用诱发天疱疮副肿瘤性天疱疮（三）线性IgA大疱性皮肤病 无密集的嗜酸性粒细胞浸润带 有微脓肿和纤维蛋白沉淀,BACK,Summary Like the skin,the oral mucosa manifests a variety of vesiculobullous diseases（大疱类疾病）.These vary in frequency,severity,and systemic consequence.The most important vesiculobullous diseases are“pemphigus”（天疱疮）and“pemphigoid”（类天疱疮）.,NEXT,Pemphigus vulgaris（寻常型天疱疮）is a serious chronic vesiculobullous disease of mucosa and skin.The disease is seen almost totally in adults over the age of 60 years.The specific cause of the disease is poorly understood.However,autoimmune reaction（自身免疫）is thought to be important in the pathogenesis of it.,NEXT,An autoimmune mechanism is suggested by the finding of antibodies in the lesions of skin and mucasa localized in the intercellular（细胞间）spaces between epithelial cells.The presence of antidesmosomal antibodies（抗桥粒抗体）results in the dissolution of intercellular cementing substance（细胞间粘合物质）.This process results in intraepithelial（上皮内）separation of the stratum germinativum（基底层）from the underlying stratum spinosum（棘层）,with subsequent acantholysis（棘层松解）and loss of epithelial integrity.,NEXT,Oral lesions are present in almost all cases of pemphigus vulgaris and often precede skin involvement.Any area of the mouth may be involved,including mucosal surfaces and gingivae.In severe cases almost the entire oral mucasa may be affected,with vesiculobullous lesions in various stages of development,regression,and healing.,NEXT,The skin lesions are initially vesiculobullous eruptions that subsequently ulcerate and are then prone to secondary infection and poor healing.Diagnosis of the disease is based on history,clinical examination,cytologic smears（细胞学涂片）,biopsy,and immunofluorescent（免疫荧光）techniques.,NEXT,Although there is no cure for pemphigus vulgaris,the disease can usually be successfully controlled with immunosuppressive drugs such as prednisone（强的松）.But these drugs may produce significant side effects.Local oral symptoms can be controlled with palliative rinses or ointment,such as dyclonine（达克罗宁）and benzocaine（苯佐卡因）in orabase.,NEXT,Pemphigoid(类天疱疮)includes cicatricial pemphigoid and bullous pemphigoid.They are also autoimmune diseases.Unlike pemphigus,the antibodies are demonstrated in the area of the epithelial basement membrane.Consequently,subepithelial vesiculobullous lesion arise,in which separation is noted between the epithelium and the underlying connective tissue.,NEXT,本课小结,天疱疮的发病机制天疱疮和类天疱疮的临床分型及其表现特征天疱疮的病理基础口腔粘膜大疱类疾病的皮肤损害，建立粘膜皮肤联发疾病概念鉴别诊断皮质激素在天疱疮和类天疱疮治疗中的应用方法、效果、注意事项,BACK,天疱疮pemphigus,NEXT,天疱疮pemphigus,NEXT,天疱疮pemphigus,揭皮试验(),BACK,天疱疮pemphigus,BACK,BACK,增殖性天疱疮Pemphigus vegetans,BACK,棘层松解（400）acantholysis,BACK,上皮内疱（120）Intraepithelial bulla,BACK,棘层松解细胞（2000）Acantholytic cell,BACK,上皮下疱（100）Subepithelial bulla,BACK,天疱疮免疫荧光Immunofluorescence of specimen from the lesions of pemphigus vulgaris,BACK,类天疱疮免疫荧光Immunofluorescence of specimen from the lesions of pemphigoid,口腔 龈：剥脱性龈炎 尼氏征()悬雍垂、软腭、扁桃体、舌腭弓等瘢痕性 粘连 畸形眼 单纯性结膜炎睑球粘连 泪管阻塞皮肤 好发于面部 头皮 水疱 疱壁厚 不易破 尼氏征()其他 咽 气管 尿道 生殖器 肛门 瘢痕粘连,BACK,瘢痕性类天疱疮,BACK,尼氏征，即尼科尔斯基（Nikolsky）征 由于天疱疮是棘层松解的结果，因此用手指轻推外表正常的皮肤与粘膜，即可迅速形成水疱，或使原有的水疱在皮肤上扩展。这种现象称为尼氏征阳性。,BACK,激素禁忌证：高血压 糖尿病 骨质疏松 消化道溃疡 各种感染 孕妇 激素不良反应：Cushings syndrome 糖尿 胃肠溃疡出血 感染扩散,BACK,
