大疱类疾病PPT.ppt

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 大疱类天疱疮 瘢痕类天疱疮 寻常型天疱疮 大疱性表皮松解症 病因 临床 病理 预后 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 vesi