黏膜医学教学（中山大学）大疱类疾病-basic-EN.pptxVIP

Bullous diseases of oral mucosa;Bullous disease of oral mucosa;What is Acantholysis? the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes;How does Acantholysis Happen? autoantibodies form against desmoglein (桥粒芯蛋白). Desmoglein forms the glue that attaches adjacent epithelium cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epithelium becomes unglued”.;How to Dignosis Acantholysis ? Nikolsky’s sign -gentle lateral pressure on unaffected mucosa or skin with a cotton swab or finger raises a bulla. 揭皮试验(+)：撕去或提取疱壁时，连同临近外观正常的黏膜/皮肤一并无痛性撕去，并遗留下一鲜红的创面 探针试验(+)：在糜烂面的边缘将探针轻轻置入黏膜下方，可见探针无痛性无阻力伸入到上皮下方或邻近的黏膜表层下方 In pemphigoid, Nikolsky sign is negative ;揭皮试验;Bullous disease of oral mucosa;Pemphigus;Clinical Features;Blisters would not show spontaneous recession Nikolsky’s sign (+) Fragile bulla, almost always ruptured by the time of diagnosis little or no erythematous inflammatory halo and a fresh lesion may retain epithelial tags at its periphery;Lab Examinations;Pathological exam;Cytological test;Direct immunofluorescence (DIF);Indirect immunofluorescence (IIF);Diagnosis;Pemphigus;Pemphigus;Clincal Features;Clincal Features;Differential Diagnosis; Treatment;Treatment –Systemic medication;Recommended dosage of Prednisone for oral pemphigus vulgaris in patients who have not yet developed skin lesions ;Treatment –topical medication;Pemphigoid;Clinical Features – mucosal lesion;Clinical Features – mucosal lesion;Clinical Features – Skin lesion;Differential Diagnosis;Differential diagnosis between CP and erosive OLP;Treatment;Treatment;Thank you!