BỘ GIÁO DỤC VÀ ĐÀO TẠO BỘ Y TẾ ĐẠI HỌC Y DƯỢC THÀNH PHỐ HỒ CHÍ MINH -oOo - LÊ THỊ ÁI NHÂN NGHIÊN CỨU ĐẶC ĐIỂM GIẢI PHẪU BỆNH VÀ MIỄN DỊCH HUỲNH QUANG TRỰC TIẾP TRONG CHẨN ĐỐN BỆNH DA BĨNG NƯỚC TỰ MIỄN LUẬN VĂN BÁC SĨ NỘI TRÚ THÀNH PHỐ HỒ CHÍ MINH – NĂM 2021 BỘ GIÁO DỤC VÀ ĐÀO TẠO BỘ Y TẾ ĐẠI HỌC Y DƯỢC THÀNH PHỐ HỒ CHÍ MINH -oOo - LÊ THỊ ÁI NHÂN NGHIÊN CỨU ĐẶC ĐIỂM GIẢI PHẪU BỆNH VÀ MIỄN DỊCH HUỲNH QUANG TRỰC TIẾP TRONG CHẨN ĐỐN BỆNH DA BĨNG NƯỚC TỰ MIỄN NGÀNH: GIẢI PHẪU BỆNH MÃ SỐ: LUẬN VĂN BÁC SĨ NỘI TRÚ NGƯỜI HƯỚNG DẪN KHOA HỌC TS.BS TRẦN HƯƠNG GIANG THÀNH PHỐ HỒ CHÍ MINH – NĂM 2021 LỜI CAM ĐOAN Tơi xin cam đoan cơng trình nghiên cứu khoa học độc lập thân chỉa dẫn người hướng dẫn khoa học Các nội dung nghiên cứu trình bày luận văn không chép từ đâu Số liệu kết nghiên cứu hoàn toàn trung thực chưa sử dụng cơng bố cơng trình khác MỤC LỤC DANH MỤC TỪ VIẾT TẮT i DANH MỤC ĐỐI CHIẾU THUẬT NGỮ VIỆT- ANH iii DANH MỤC BẢNG v DANH MỤC BIỂU ĐỒ vii DANH MỤC CÔNG THỨC viii DANH MỤC SƠ ĐỒ .ix DANH MỤC HÌNH x ĐẶT VẤN ĐỀ CHƯƠNG 1: TỔNG QUAN TÀI LIỆU 1.1 Đại cương mô học da 1.1.1 Lớp thượng bì 1.1.2 Lớp bì 1.1.3 Lớp hạ bì 1.2 Khái quát BDBNTM 1.2.1 Định nghĩa phân loại BDBNTM 1.2.2 Cơ chế bệnh sinh BDBNTM 1.3 Nhóm bệnh Pemphigus 12 1.3.1 Pemphigus thông thường 12 1.3.2 Pemphigus (PF) 14 1.3.3 Pemphigus sùi (Pemphigus vegetens- PVe) 16 1.3.4 Pemphigus đỏ da (Pemphigus erythematosus- PE) 16 1.3.5 Pemphigus đặc hữu (Endemic pemphigus foliaceus- EPF) 17 1.3.6 Pemphigus IgA (Immunoglobulin A pemphigus- IAP) 18 1.3.7 Pemphigus cận u (Paraneoplastic pemphigus- PNP) 19 1.3.8 Pemphigus dạng herpes (Pemphigus herpetiformis- PH) 21 1.3.9 Pemphigus thuốc (DIP) 22 1.4 Nhóm pemphigoid 22 1.4.1 Pemphigoid bóng nước (Bullous pemphigoid- BP) 22 1.4.2 Pemphigoid niêm mạc (Mucous membrane pemphigoid-MMP) 25 1.4.3 Pemphigoid thai kỳ (Pemphigoid gestationis) 26 1.4.4 Ly thượng bì bóng nước mắc phải (EBA) 27 1.4.5 Bệnh IgA đường (Linear IgA disease- LAD) 28 1.4.6 Lupus ban đỏ hệ thống thể bóng nước (BSLE) 30 1.5 Viêm da dạng herpes (Dermatitis herpetiformis – DH) 32 1.6 Xét nghiệm DIF 33 1.6.1 Nguyên lý chung xét nghiệm DIF 33 1.6.2 DIF chẩn đốn bệnh da bóng nước tự miễn 34 CHƯƠNG 2: ĐỐI TƯỢNG VÀ PHƯƠNG PHÁP NGHIÊN CỨU 43 2.1 Đối tượng nghiên cứu 43 2.1.1 Tiêu chuẩn chọn bệnh vào mẫu nghiên cứu 43 2.1.2 Tiêu chuẩn loại trừ 46 2.2 Phương pháp nghiên cứu 46 2.2.1 Thiết kế nghiên cứu 46 2.2.2 Cách chọn mẫu nghiên cứu 46 2.2.3 Tiến trình nghiên cứu 46 2.3 Các biến số nghiên cứu 49 2.3.1 Các biến số mẫu nghiên cứu 49 2.3.2 Các biến số đặc điểm giải phẫu bệnh tổn thương 49 2.3.3 Các biến số đặc điểm lắng đọng KT tiêu nhuộm DIF 52 2.3.4 Chẩn đoán mô bệnh học 53 2.3.5 Chẩn đoán DIF 54 2.3.6 Chẩn đoán xác định 55 2.4 Y đức nghiên cứu 56 2.5 Thời gian địa điểm tiến hành nghiên cứu 56 CHƯƠNG 3: KẾT QUẢ NGHIÊN CỨU 57 3.1 Một số đặc điểm lâm sàng bệnh da bóng nước tự miễn 57 3.1.1 Tỷ lệ số lượng BDBNTM 57 3.1.2 Giới tính bệnh nhân 58 3.1.3 Tuổi bệnh nhân 59 3.2 Đặc điểm giải phẫu bệnh BDBNTM 60 3.2.1 Đặc điểm đại thể tổn thương da BDBNTM 60 3.2.2 Đặc điểm vi thể tổn thương tiêu H&E 64 3.3 Đặc điểm lắng đọng kháng thể DIF BDBNTM 69 3.3.1 Loại KT lắng đọng 69 3.3.2 Vị trí lắng đọng KT 71 3.3.3 Kiểu lắng đọng KT 72 3.3.4 Cường độ lắng đọng KT 73 3.4 Đánh giá tương hợp lâm sàng, mô bệnh học DIF chẩn đoán BDBNTM 76 3.4.1 Sự tương hợp lâm sàng, mô bệnh học DIF chẩn đoán BDBNTM 77 3.4.2 Sự tương hợp lâm sàng, mơ bệnh học DIF chẩn đốn bệnh nhóm pemphigus 77 3.4.3 Sự tương hợp lâm sàng, mô bệnh học DIF chẩn đốn BDBNTM thượng bì 80 CHƯƠNG 4: BÀN LUẬN 83 4.1 Một số đặc điểm lâm sàng bệnh da bóng nước tự miễn 83 4.1.1 Tỷ lệ số lượng BDBNTM 83 4.1.2 Giới tính bệnh nhân 86 4.1.3 Tuổi bệnh nhân 88 4.2 Đặc điểm giải phẫu bệnh BDBNTM 92 4.2.1 Đặc điểm đại thể tổn thương BDBNTM 92 4.2.2 Đặc điểm vi thể tổn thương tiêu H&E 101 4.3 Đặc điểm lắng đọng kháng thể DIF BDBNTM 107 4.3.1 Loại KT lắng đọng 107 4.3.2 Vị trí lắng đọng KT 113 4.3.3 Kiểu lắng đọng KT 115 4.3.4 Cường độ lắng đọng KT 117 4.4 Đánh giá tương hợp lâm sàng, mô bệnh học DIF chẩn đoán BDBNTM 119 4.4.1 Sự tương hợp lâm sàng, mơ bệnh học DIF chẩn đốn BDBNTM 119 4.4.2 Sự tương hợp lâm sàng, mô bệnh học DIF chẩn đốn bệnh nhóm pemphigus 122 4.4.3 Sự tương hợp lâm sàng, mô bệnh học DIF chẩn đốn BDBNTM thượng bì 125 CHƯƠNG 5: KẾT LUẬN 130 5.1 Một số đặc điểm lâm sàng bệnh da bóng nước tự miễn 130 5.2 Đặc điểm giải phẫu bệnh DIF BDBNTM 130 5.2.1 Đặc điểm đại thể tổn thương BDBNTM 130 5.2.2 Đặc điểm vi thể tổn thương BDBNTM 131 5.2.3 Đặc điểm DIF 131 5.3 Sự tương hợp LS, MBH DIF chẩn đoán BDBNTM 132 CHƯƠNG 6: KIẾN NGHỊ 133 TÀI LIỆU THAM KHẢO 134 i DANH MỤC TỪ VIẾT TẮT TÊN VIẾT TẮT TÊN ĐẦY ĐỦ BCAT Bạch cầu toan BCĐNTT Bạch cầu đa nhân trung tính BDBNTM Bệnh da bóng nước tự miễn BN Bệnh nhân BP Pemphigoid bóng nước BSLE Lupus ban đỏ hệ thống thể bóng nước DH Viêm da dạng herpes DIP Pemphigus thuốc Dsg Desmoglein EBA Ly thượng bì bóng nước mắc phải ELISA Điện di hấp phụ miễn dịch gắn men EPF Pemphigus đặc hữu GP Pemphigoid thai kỳ GSE Bệnh đường ruột nhạy cảm gluten HMMD Hố mơ miễn dịch KHV Kính hiển vi ii KT Kháng thể LAD Bệnh IgA đường LPP Lichen phẳng thể bóng nước DIF Miễn dịch huỳnh quang trực tiếp MMP Pemphigoid niêm mạc PE Pemphigus đỏ da PF Pemphigus PH Pemphigus dạng herpes IAP Pemphigus IgA PNP Pemphigus cận ung Pve Pemphigus sùi PV PV TB Tế bào iii DANH MỤC ĐỐI CHIẾU THUẬT NGỮ VIỆT- ANH TỪ TIẾNG VIỆT TỪ TIẾNG ANH Bệnh IgA đường Linear IgA disease Điện di miễn dịch gắn men Enzyme-Linked Immunosorbent Assay Lichen phẳng thể bóng nước Lichen planus pemphigoid Lupus ban đỏ hệ thống thể bóng nước Bullous systemic lupus erythematous Ly thượng bì bóng nước mắc phải Epidermolysis bullosa acquisita Pemphigoid bóng nước Bullous pemphigoid Pemphigoid niêm mạc Mucous membrane pemphigoid Pemphigoid thai kỳ Gestational pemphigoid Pemphigus cận ung Paraneoplastic pemphigus Pemphigus đặc hữu Endemic pemphigus foliaceus Pemphigus dạng herpes Pemphigus herpetiformis Pemphigus đỏ da Pemphigus 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chẩn đoán bệnh da bóng nước tự miễn? ?? nhằm mục tiêu: Mô tả số đặc điểm lâm sàng bệnh da bóng nước tự miễn Khảo sát đặc điểm giải phẫu bệnh DIF số BDBNTM... bệnh học chẩn đốn BDBNTM, chúng tơi đặt câu hỏi “Các đặc điểm giải phẫu bệnh DIF có vai trị chẩn đốn bệnh da bóng nước tự miễn? ” Vì chúng tơi thực đề tài ? ?Nghiên cứu đặc điểm giải phẫu bệnh miễn. .. DỤC VÀ ĐÀO TẠO BỘ Y TẾ ĐẠI HỌC Y DƯỢC THÀNH PHỐ HỒ CHÍ MINH -oOo - LÊ THỊ ÁI NHÂN NGHIÊN CỨU ĐẶC ĐIỂM GIẢI PHẪU BỆNH VÀ MIỄN DỊCH HUỲNH QUANG TRỰC TIẾP TRONG CHẨN ĐỐN BỆNH DA BĨNG NƯỚC TỰ MIỄN