Toxic Epidermal Necrolysis/Stevens-Johnson Syndrome at A University Hospital in Saudi: Causative Factors and Outcomes

Introduction:Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare, life-threatening conditions caused mainly by drugs. Their management relies on the withdrawal of the culprit medication and supportive measures. Different pharmacotherapies have varied effects. However, data related to TEN and SJS in Saudi is limited. This study aimed to identify the causative agents, associated factors, and outcomes of TEN/SJS cases admitted to a teaching hospital (King Abdulaziz University) in Jeddah during the last 10 years.Methods: We retrospectively analyzed the data of TEN/SJS patients admitted to the hospital over the last 10 years.Results: We identified 12 patients with TEN/SJS. Of these, nine survived the condition and were discharged. The culprit medication was identified in eight of them, including antibiotics in six cases and Tegretol and allopurinol in one case each. Most of the patients received systemic steroids and intravenous immunoglobulins.Conclusion: TEN/SJS is mainly caused by medications of which antibiotics are the most implicated. Consistent with other studies, the mortality rate associated with TEN/SJS in Saudi is 25%. Limitations: restricted to a single center and small sample size.

KẾT QUẢ CỦA GLOBULIN MIỄN DỊCH TRUYỀN TĨNH MẠCH TRONG ĐIỀU TRỊ HOẠI TỬ THƯỢNG BÌ NHIỄM ĐỘC DO THUỐC

Mục tiêu: Nghiên cứu nhằm đánh giá kết quả điều trị của globulin miễn dịch truyền tĩnh mạch (IVIg) trong điều trị hoại tử thượng bì nhiễm độc (Toxic epidermal necrolysis - TEN) do thuốc. Phương pháp nghiên cứu: Nghiên cứu hồi cứu trên 29 bệnh nhân chẩn đoán TEN nghi do thuốc tại Trung tâm Dị ứng - Miễn dịch lâm sàng, Bệnh viện Bạch Mai từ tháng 4 năm 2019 đến tháng 7 năm 2020. Kết quả: Trong tổng số 29 bệnh nhân, IVIg được sử dụng điều trị trong 9 trường hợp. Tỉ lệ tử vong trong nhóm sử dụng IVIg là 0% so với 10% ở nhóm không điều trị IVIg. Thời gian nằm viện trung bình của nhóm có sử dụng IVIG là 15,33 ± 7,632 ngày không có sự khác biệt với nhóm không điều trị IVIg là 14,15 ± 4,392 ngày. Kết luận: IVIg là một trong số các phương pháp điều trị toàn thân được đưa vào sử dụng trong điều trị TEN, tuy nhiên hiệu quả điều trị còn nhiều tranh cãi, cần có những nghiên cứu lớn và toàn diện hơn để đánh giá hiệu quả của phương pháp này.

Differential Diagnostics of Toxic Epidermal Necrolysis (Lyell’s Syndrome) in ICU: Case Reports

Toxic epidermal necrolysis (TEN) is a critical life-threating condition developing as the total detachment of epidermidis and characterized by severe pathological reactions of all body systems. The current article describes two cases of TEN with similar clinical and laboratory signs. In one case the diagnosis of TEN was subsequently refused.The objective: analysis of methods of clinical and differential diagnostics of conditions accompanied with massive epidermidis detachment in ICU patients.Results. The immunomorphological evaluation of skin specimen obtained from the patient with a torpid form of TEN showed linear IgG fixation in the intercellular space of stratum basale, stratum spinosum and stratum granulosum and C3 fixation in the intercellular space of stratum basale.Conclusion. The complex of anamnesis data and pathomorphological evaluation of skin are crucial for the diagnosis and treatment of patients with atypical TEN.

Toxic epidermal necrolysis associated with anticonvulsants

Background and Objetives: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as a continuum of the same process. TEN or Lyell Syndrome is the most severe form. Both entities involve an acute mucocutaneous blistering reaction associated with systemic inflammation. Materials and Methods: We present a case of a young woman who developed TEN following concomitant treatment with valproate, lamotrigine, and phenobarbital. Despite the extensive mucocutaneous detachment (over 90%), prognostic evaluation was favorable (SCORTEN score 2; probability of survival 88%), and this patient evolved satisfactorily. Five days after admission, valproate was reinitiated without any subsequent adverse reaction. Results: Causality evaluation identified both lamotrigine and phenobarbital as “very probable” (ALDEN score = 6) causes and valproate as “very unlikely” (ALDEN score = 0) cause of TEN. Conclusions: SJS and TEN are true life-threatening medical emergencies. This case emphasizes the importance of early diagnosis and treatment, including the discontinuation of the causative agent, which can be lifesaving.