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Những Quan Điểm Hiện Tại Về Hội Chứng Stevens-Johnson và Hoại Tử Biểu Bì Độc
Tóm tắt
Hội chứng Stevens-Johnson (SJS) và hoại tử biểu bì độc (TEN) được xem xét như là một phản ứng quá mẫn độ trễ do thuốc gây ra. Chúng đại diện cho những tình huống y tế cấp cứu thực sự, và việc nhận diện sớm cùng với quản lý phù hợp là quyết định cho sự sống còn. SJS/TEN biểu hiện với một giai đoạn tiền triệu giống như "cúm" (mệt mỏi, sốt), tiếp theo là tổn thương đau đớn trên da và màng nhầy (mắt, miệng, và bộ phận sinh dục), kèm theo các triệu chứng hệ thống khác. Sự khác biệt giữa SJS, SJS/TEN chồng lắp, và TEN được xác định bởi mức độ tách lớp da: SJS được định nghĩa là tổn thương da < 10%, TEN được định nghĩa là tổn thương da > 30%, và SJS/TEN chồng lắp là tổn thương da từ 10–30%. Việc chẩn đoán các mức độ khác nhau của hoại tử biểu bì dựa trên đánh giá lâm sàng kết hợp với histopathology tương ứng. Tỷ lệ tử vong cho SJS và TEN đã giảm trong những thập kỷ qua. Hiện tại, thang điểm mức độ nghiêm trọng của bệnh cho hoại tử biểu bì độc (SCORTEN) có sẵn để đánh giá mức độ nghiêm trọng của SJS/TEN. Các thuốc có nguy cơ cao gây SJS/TEN bao gồm sulfonamid kháng khuẩn, thuốc chống động kinh, thuốc chống viêm không steroid loại oxicam, allopurinol, nevirapine, và chlormezanone. Ngoài các thuốc truyền thống, các phương pháp chữa bệnh bằng thảo dược và các chế phẩm sinh học mới cũng cần được xem xét như là các tác nhân gây bệnh. Nguy cơ gia tăng các phản ứng dị ứng với một số loại thuốc có thể liên quan đến các kháng nguyên HLA đặc hiệu. Hiểu biết của chúng ta về sinh bệnh học của SJS/TEN đã được cải thiện: sự độc tế bào T đặc hiệu thuốc, liên kết di truyền với các gen HLA và không HLA, hạn chế TCR và các cơ chế độc. Tuy nhiên, nhiều yếu tố góp phần vào hoại tử biểu bì vẫn cần được xác định, đặc biệt là trong các dạng hoại tử biểu bì do virus gây ra và tự miễn không liên quan đến thuốc. Trong SJS/TEN, các biến chứng phổ biến nhất là mắt, da, hoặc thận. Sự tham gia của màng nhầy hầu họng, thực quản, và bộ phận sinh dục với các bọng nước, loét cũng như sự phát triển thứ cấp của các chít hẹp cũng đóng vai trò. Tuy nhiên, trong giai đoạn cấp tính, nhiễm trùng huyết là nguyên nhân hàng đầu của bệnh tật và tỷ lệ tử vong. Sự tham gia của phổi và gan là thường xuyên. Quản lý cấp tính SJS/TEN yêu cầu một phương pháp đa ngành. Việc ngừng ngay lập tức các thuốc có khả năng gây hại là điều bắt buộc. Việc giới thiệu nhanh chóng đến một trung tâm y tế thích hợp để điều trị hỗ trợ cụ thể là cực kỳ quan trọng. Các điều trị thường được sử dụng cho SJS/TEN là corticosteroid toàn thân, immunoglobulin và cyclosporin A.
Từ khóa
#Hội chứng Stevens-Johnson #hoại tử biểu bì độc #quá mẫn độ trễ #điều trị đa ngành #SCORTENTài liệu tham khảo
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