SJS.TEN .pdf

SJS.TEN .pdf
SJS /TEN SJS /TEN BS.NTH BS.NTH
Clinical case Bé trai, 5 tuổi, nhập viện vì sốt Quá trình bệnh lý : N1-2: bé sốt cao , có loét miệng và lưỡi, khám pk tư đk điều trị vs thuốc chích không rõ loại ( vitC, gentamycine, nước cất??), triệu chứng có giảm . N3-N4 : em xuất hiện đỏ mắt tiết gèn nhiều, kèm nổi hồng ban rải rác vùng cổ, sau đó chuyển thành mụn nước, tự vỡ, sau vỡ để lại vệt trợt, kèm sốt cao, điều trị tại BV tuyến tỉnh không đỡ=> BVNĐ 1.
PHÂN LOẠI • SJS is the less severe condition, in which skin detachment is <10 percent of the body surface (picture 1A-C). • TEN involves detachment of >30 percent of the body surface area (BSA) (picture 2A-D). • SJS/TEN overlap describes patients with skin detachment of 10 to 30 percent of BSA. • We will use the term "SJS/TEN" to refer collectively to SJS, TEN, and SJS/TEN overlap syndrome. SJS : <10 % BSA TEN > 30% BSA SJS/TEN 10-30 % BSA
NGUYÊN NHÂN • Thuốc • Mycoplasma pneumoniae infection • Khác -Infections, including Mycoplasma pneumoniae infection, are the next most common trigger of SJS/TEN, particularly in children [31-33] -In over one-third of SJS/TEN cases, no cause can be identified
YTNC • Nhiễm HIV, gen, bệnh miễn dịch, bệnh ác tính, yếu tố vật lý ( UV, phóng xạ) • Genetic factors : Carbamazepine HLH-B*15:02 • Khi nào sàng lọc di truyền ?
• Type IV • Phản ứng type IV không qua kháng thể mà qua T cells. • T cells, sensitized after contact with a specific antigen, are activated by continued exposure or reexposure to the antigen; they damage tissue by direct toxic effects or through release of cytokines, which activate eosinophils, monocytes and macrophages, neutrophils, or natural killer cells. • Disorders involving type IV reactions include Stevens- Johnson syndrome, toxic epidermal necrolysis (SJS/TEN), drug rash with eosinophilia and systemic symptoms (DRESS) , contact dermatitis (eg, poison ivy), subacute and chronic hypersensitivity pneumonitis, acute and chronic allograft rejection, the immune response to tuberculosis, and many forms of drug hypersensitivity.
-DHS/DRESS -AGEP -SJS/TEN
CHẨN ĐOÁN : • no universally accepted diagnostic criteria for Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), and histologic findings are neither specific nor diagnostic. Despite these limitations, the diagnosis of SJS or TEN would be appropriate in a patient with the following clinical features:
CHẨN ĐOÁN • Bệnh sử & khám lâm sàng cẩn thận • Đánh giá thuốc tiếp xúc : tên thuốc, thời gian uống.. • Đánh giá mức độ nặng của bệnh • Sinh thiết da • XN CLS & hình ảnh học : CTM, Glucose, ĐGĐ, ure, cre, Calcium, protein, Albumin, ALP, AST, ALP, VS, CRP , cấy máu, dịch tiết tại vị trí tổn thương,PCR và/ hoặc huyết thanh M. pneumoniae, X-ray.
BIẾN CHỨNG ? • Biến chứng cấp : mất nước, điện giải, shock giảm thể tích gây suy thận, NKH, kháng insuline ,tăng chuyển hóa ,MODS Pulmonary complications Gastrointestinal complications Infection Disseminated intravascular coagulation ❑Nhiễm trùng : Sepsis & septic shock do Staphylococcus aureus and Pseudomonas aeruginosa => nguyên nhân chính gây tử vong. ❑Biến chứng tại phổi : VP, VP kẽ ❑Biến chứng tiêu hóa : tiêu chảy, tiêu phân đen, loét ruột non, thủng đại tràng, lồng ruột non. ❑DIC : 150 BN SJS/TEN , 32 BN ( 21 %) có DIC trên xét nghiện => xuất huyết tiêu hóa, hô hấp, thận, suy gan, nhiễm trùng, NTH, và tử vong.
CHẨN ĐOÁN PHÂN BIỆT Hồng ban đa dạng (Erythema multiforme) Đỏ da (Erythroderma) và hồng ban do thuốc . Hội chứng mụn mủ ngoại ban toàn thân cấp tính (AGEP) Phát ban cố định thuốc thể bọng nước lan tỏa (Generalized Bullous Fixed Drug Eruption) Hồng ban do phản ứng ánh sáng Staphylococcal scalded skin syndrome Paraneoplastic pemphigus Bệnh IgA bọng nước thành dải (LABD) Chikungunya fever
ĐIỀU TRỊ PHASE CẤP PHASE MẠN 5-7 days Bệnh sinh cutaneous detachment mucositis disease arrest and re-epithelization Biến chứng Quản lý hồi phục fluid and electrolyte imbalances, sepsis, organ decompensation, and death supportive care and prevention of short- and long-term complications Physical and psychologic sequelae screening and treatment of these complications in order to maintain life quality
PHASE CẤP Điều trị hỗ trợ : + Chăm sóc vết thương + Hiệu chỉnh điện giải, bù dịch + Dinh dưỡng + Nhiệt độ + Kiểm soát đau +Dự phòng và điều trị nhiễm trùng + Chăm sóc mắt + Hỗ trợ các cơ quan khác nếu bị ảnh hưởng.
DỊCH &ĐIỆN GIẢI & DINH DƯỠNG • Tăng mất nước qua da và giảm nhập đường miệng • Theo dõi chặt chẽ bilan xuất-nhập • Bù dịch để ngừa giảm tưới máu (end-organ hypoperfusion ) và shock • Thể tích dịch cần bù ? Trong 24 h đầu , nhu cầu dịch 2mL/kg/BSA bị ảnh hưởng , sau đó dịch nên được hiệu chỉnh theo đáp ứng của bệnh nhân và lượng nước tiểu, mục tiêu V nước tiểu : 0.5 -1 mL/kg/giờ • Điều chỉnh điện giải theo kết quả CLS . • Nhu cầu 20-25 kcal/kg/ngày trong phase sớm , phase chuyển hóa of SJS/TEN và 25-30 kcal/kg/ngày trong phase hồi phục .
ADJUNCTIVE PHARMACOLOGIC THERAPIES • Cyclosporine • Etanercept • Systemic corticosteroids • Combination of IVIG and systemic corticosteroids
Systemic corticosteroids -Prednisolone 60 -250 mg/ngày x 2 -12 days, -Dexamethasone IV 1.5 mg/kg/ngày x 3 ngày - Methylprednisolone IV 250 -1000 mg/day x3 ngày efficacy of systemic corticosteroids has not been proven. Thus, routine use of systemic corticosteroids cannot be recommended Cyclosporine 3 -5 mg/kg/ngày trong 24-48h khởi phát triệu chứng -reduced mortality -use of cyclosporine early in the course of the disease has consequently been advocated by some experts Intravenous immune globulin a dose of 2 to 4 g/kg over two to five days initially proposed as a treatment for SJS/TEN based on the hypothesis that Fas ligand (FasL) was the main mediator of widespread keratinocyte apoptosis in TEN and on the finding that high-dose IVIG was able to antagonize FasL effects [95]. However, it is now widely accepted that granulysin, a cytotoxic protein found in cytotoxic T cells, is the most important mediator
Kumar R, Das A, Das S. Management of Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: Looking Beyond Guidelines! Indian J Dermatol. 2018 Mar-Apr;63(2):117-124. doi: 10.4103/ijd.IJD_583_17. PMID: 29692452; PMCID: PMC5903040.

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