醫學筆記匯整 ED Notes

• Steven Johnson Syndrome
• Toxic Epidermal Necrosis
  
  10 ~ 30 % 叫做 SJS-TEN overlap
  
  出現症狀前八周內的藥物都有可能，平均為用藥後兩周
   

   


* 現在不太提 EM major, EM 和 SJS/TEN 被認為是兩種疾病
   不需要強行要區分兩者, 反正都還是要停藥
   SJS/TEN 的 epidermis 要分離, 會很痛
   以癢為主要表現的比較不用擔心, 會往 EM major  想

 

• Immune complex mediated hypersensitivity
   
• Allopurinol : HLA-B 5801
• Carbamazepine and phenytoin induced SJS
  is strongly associated with HLA-B 1502
   
• Typically involves skin and mucous membranes
  皮膚廣泛性脫落壞死 + 黏膜糜爛等不良反應
   
• 臨床特徵包括了特有的皮疹和黏膜侵犯
  皮疹 為典型的圓環狀 Target lesion
  黏膜則有兩處以上之侵犯 ( 口, 鼻, 眼, 生殖器, 肛門 )
  會嚴重波及身體許多器官如：肺, 肝, 腎, 腸胃, 血液系統時
  造成體液喪失, 體溫調節失調, 代謝率增加
  
  
   

* Clinical Course : 

1. Typical prodromal symptoms 類流感前驅症狀 :
   Fever (大多>39C) and influenza like symptoms 最早出現
   在疹子發生前 1~3 天
   
   - Mild fever, Headache
   - Productive Cough, Sore throat
   - Malaise
   - Arthralgia
   - Pruritis
   - Red eyes, Photophobia and conjunctival itching or burning, and pain on swallowing
     ( 早期 mucosal involvement 的症狀 )
    
2. Rash ( Pain + Burning )
   symmetrically on face and the upper part of the trunk
   
   皮膚紅疹一開始常常是刺痛, 灼熱的皮膚發疹
   呈現對稱性擴展到全身
   一開始的皮膚病灶只是境界不鮮明的紅斑, 伴有中央的紫黑色斑
   隨後在短短幾天內演變成全身大面積的紅斑
   每個病灶中央部位則呈現壞死潰爛的情形, 稱做標靶樣病灶
   
   呈現出 :
   
   Erythema multiforme ( Target lesion )
                             or
   Maculo-papular ( Erythema morbilliform ) 
   
    
3. Erosion of mucus membrane ( > 2 sites )
    
4. 最後進展到很多 Epidermal detachment ( 跟嚴重傷燒一樣 )
   可能有許多 blister 
   Skin involement 區域只要有 detachment 都算在面積裡
   
   
    

• Oral ulcer 很痛無法進食
• Conjunctivitis when with eye involvement
• Esophageal strictures ( GI mucosa )
• 續發 Infection
• 造成 Volume loss (hypovolemic), Electrolyte imbalance 
• 體溫失調, 代謝率增加
  
   

   

* Lab : 
- Anemia and lymphopenia are common
- If massive transdermal fluid loss and hypercatabolic state :
   hypoalbuminemia, electrolyte imbalance,
   increased blood urea nitrogen and glucose ( severe SJS/ TEN )

* Prognosis :
   Mortality rate: SJS 10%, TEN >40%


 

Backtar 也很惡名昭彰
 

• 最重要且最常見的原因是 Drug
  很少很少數才是 Infection, Cancer
  
   
• Mycoplasma pneumonia 和 CMV, 多數造成在小孩身上的 SJS
  - mucous membrane >> skin involve
  
   

1. 停藥
    
2. Supportive care :
   - Adequate hydration, UOP
   - Prophylactic antibiotics
   - Electrolytes : 鈉鉀鈣
   - 保溫
   - Pain control 
    
3. High dose Steroid
   - Short course of moderate to high dose of systemic corticosteroids
   - Prednisone 1 to 2 mg/kg per day for three to five days may have
      a beneficial effect if given early in the course of the disease
   - Corticosteroids theoretically increase the risk of sepsis and protein catabolism and
     decrease the rate of epithelialization
      patients with extensive skin detachment is contraindicated
   - Add 胃藥 to avoid steroid caused and stress ulcer
   
   IVIG
   - A dose of 1 g/kg per day may be given for three consecutive days
     (total dose 3 g/kg) in the early phase of the disease
   - adverse effects include renal, hematologic, and thrombotic complications

https://cdn.fbsbx.com/v/t59.2708-21/14378390_10209666995704651_5284916262265159680_n.pdf/SJS.pdf?oh=8fb904d8e626bdcc972167810922d272&oe=57EEC11C&dl=1