Balai Manisha, Meena Manju, Mittal Asit, Gupta Lalit Kumar, Khare Ashok Kumar, Mehta Sharad
Department of Dermatology, Venereology and Leprosy, R.N.T. Medical College, Udaipur, Rajasthan, India.
Department of Dermatology, Government Doon Medical College, Dehradun, Uttarakhand, India.
Indian Dermatol Online J. 2020 Sep 28;12(1):116-122. doi: 10.4103/idoj.IDOJ_326_20. eCollection 2021 Jan-Feb.
Stevens-Johnson syndrome and toxic epidermal necrolysis are severe, life-threatening mucocutaneous drug reactions with a high morbidity and mortality that require immediate medical care. Several immunomodulatory drugs are used for the treatment but evidence of their efficacy is limited. Cyclosporine has recently been found to have a promising role in SJS/TEN owing to its potent antiapoptotic activity.
This open label prospective study was conducted to determine the efficacy, safety, and tolerability of cyclosporine in patients with SJS/TEN.
This study was conducted at a tertiary care teaching hospital of South Rajasthan during a period of 4 years (August 2015 to July 2019). Data regarding clinical profile, causative drug(s), disease severity, associated comorbidities, treatment received, and outcome were recorded in a predesigned proforma. SCORTEN prognostic score was calculated for each patient at the time of admission. Cyclosporine was administered in a dose of 5 mg/kg body weight in two divided dosage until reepithelization.
Out of 16 patients 10 were males and 6 were females. Mean age of patients was 30.62 ± 16.98 years (range: 7-63). Most of the patients, i.e., 8 out of 16 had TEN, 5 patients had SJS, and 3 patients had SJS/TEN overlap. Mean ± SD delay between onset and admission was 3.812 ± 1.377 days (range: 2-7). Among the suspected drugs, antiepileptics (43.7%) formed the major group. Mean duration of reepithelization was 10.5 ± 3.46 days (range: 7-15). Based on the SCORTEN, the expected mortality was 2.55 with mean predicted mortality rate of 16.43% with SD of 19.3.
We recommend cyclosporine (5 mg/kg/day) as the first line-specific immunomodulatory agent in SJS/TEN on account of its efficacy, safety, rapid reepithelization, decrease hospital stay, and reduced morbidity and mortality.
史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症是严重的、危及生命的皮肤黏膜药物反应，发病率和死亡率高，需要立即就医。几种免疫调节药物用于治疗，但其疗效证据有限。由于环孢素具有强大的抗凋亡活性，最近发现其在史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症中具有广阔前景。
开展这项开放标签前瞻性研究，以确定环孢素对史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症患者的疗效、安全性和耐受性。
本研究在南拉贾斯坦邦的一家三级护理教学医院进行，为期4年（2015年8月至2019年7月）。有关临床特征、致病药物、疾病严重程度、相关合并症、接受的治疗及结果的数据记录在预先设计的表格中。入院时为每位患者计算SCORTEN预后评分。环孢素按5mg/kg体重分两次给药，直至上皮再形成。
16例患者中，10例为男性，6例为女性。患者平均年龄为30.62±16.98岁（范围：7-63岁）。大多数患者，即16例中的8例患有中毒性表皮坏死松解症，5例患有史蒂文斯-约翰逊综合征，3例患有史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症重叠型。发病与入院之间的平均±标准差延迟为3.812±1.377天（范围：2-7天）。在可疑药物中，抗癫痫药（43.7%）占主要群体。上皮再形成的平均持续时间为10.5±3.46天（范围：7-15天）。根据SCORTEN评分，预期死亡率为2.55，平均预测死亡率为16.43%，标准差为19.3。
1）样本量小。2）由于疾病严重程度，无法进行安慰剂对照试验。
鉴于环孢素（5mg/kg/天）的疗效、安全性、快速上皮再形成、缩短住院时间以及降低发病率和死亡率，我们推荐将其作为史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症的一线特异性免疫调节药物。
