Disseminated intravascular coagulation in Stevens-Johnson syndrome and toxic epidermal necrolysis

• Published in: Journal of the American Academy of Dermatology 2021-06, Vol.84 (6), p.1782-1791
• Main Authors: Chen, Chun-Bing, Hsu, Tsun-Hao, Chung-Yee Hui, Rosaline, Lu, Chun-Wei, Chen, Wei-Ti, Chiang, Pin-Hsuan, Wang, Chuang-Wei, Chuang, Shiow-Shuh, Yang, Jui-Yung, Yang, Shih-Yi, Chang, Shu-Ying, Hsiao, Yen-Chang, Kao, Kuo-Chin, Hu, Han-Chung, Wu, Ting-Shu, Hsu, Chao-Wei, Hui-Kang Ma, David, Chen, Shin-Yi, Tian, Ya-Chung, Cheng, Chi-Yuan, Chen, Chi-Hua, Chi, Min-Hui, Wu, Ming-Ying, Liu, Ren-Feng, Wang, Chi-Hui, Chang, Ya-Ching, Lin, Jing-Yi, Ho, Hsin-Chun, Yu-Wei Lin, Yang, Chang, Chee Jen, Lin, Yu-Jr, Ku, Cheng-Lung, Hung, Shuen-Iu, Chung, Wen-Hung
• Format: Article
• Language: English
• Subjects: coagulopathy; disseminated intravascular coagulation; severe cutaneous adverse reactions; Stevens-Johnson syndrome; thrombocytopenia; toxic epidermal necrolysis
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2020.08.065

Patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have high mortality rates. Disseminated intravascular coagulation has been reported in SJS/TEN patients. The influence of this lethal complication in patients with SJS/TEN is not well known. This study aimed to investigate the risk and outcomes of disseminated intravascular coagulation in patients with SJS/TEN. We analyzed the disseminated intravascular coagulation profiles of patients receiving a diagnosis of SJS/TEN between 2010 and 2019. We analyzed 150 patients with SJS/TEN (75 with SJS, 22 with overlapping SJS/TEN, and 53 with TEN) and their complete disseminated intravascular coagulation profiles. Disseminated intravascular coagulation was diagnosed in 32 patients (21.3%), primarily those with TEN. It was significantly associated with systemic complications, including gastrointestinal bleeding, respiratory failure, renal failure, liver failure, infection, and bacteremia. Additionally, SJS/TEN patients with disseminated intravascular coagulation had elevated procalcitonin levels. Among patients with SJS/TEN, disseminated intravascular coagulation was associated with a greater than 10-fold increase in mortality (78.1% vs 7%). The study limitations include small sample size and a single hospital system. Disseminated intravascular coagulation is a potential complication of SJS/TEN and associated with higher mortality. Early recognition and appropriate management of this critical complication are important for patients with SJS/TEN.