Bechek Sophia, Garcia Manuel, Chiou Howard
Stanford University School of Medicine, Stanford, CA, USA.
Division of Pediatric Gastroenterology, Stanford University School of Medicine, Stanford, CA, USA.
Clin Exp Gastroenterol. 2020 Sep 30;13:377-383. doi: 10.2147/CEG.S269349. eCollection 2020.
Stevens-Johnson syndrome and toxic epidermal necrolysis form a rare but severe disease spectrum characterized by widespread epidermal detachment. Gastrointestinal manifestations of the disease, however, are rarely described in the pediatric literature and have a high mortality among adults. There are limited data on the treatment of these cases, with conflicting evidence regarding the benefit of steroids, IVIG, or other immunosuppressive agents. We review previous instances of gastrointestinal involvement in children and report the case of a previously healthy 13-year-old who presented with the typical ocular and skin findings of Stevens-Johnson syndrome, subsequently developed severe life-threatening diarrhea, and was found to have severe esophagitis, duodenitis, and colitis on endoscopic evaluation. Treatment was initiated with an immediate, short course of steroids along with early introduction of an enteral diet via nasogastric tube, and resulted in full gastrointestinal recovery. This case highlights successful medical treatment of the first reported pediatric case of SJS/TEN with both upper and lower gastrointestinal tract involvement.
史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症构成一种罕见但严重的疾病谱,其特征为广泛的表皮剥脱。然而,该疾病的胃肠道表现很少在儿科文献中被描述,且在成人中具有高死亡率。关于这些病例的治疗数据有限,对于类固醇、静脉注射免疫球蛋白或其他免疫抑制剂的益处存在相互矛盾的证据。我们回顾了既往儿童胃肠道受累的病例,并报告了一名此前健康的13岁儿童的病例,该患儿出现了史蒂文斯-约翰逊综合征典型的眼部和皮肤表现,随后发展为严重的危及生命的腹泻,在内镜评估中发现患有严重食管炎、十二指肠炎症和结肠炎。治疗立即开始使用短期类固醇疗程,并通过鼻胃管尽早引入肠内营养,最终实现了胃肠道的完全恢复。本病例突出了首例报道的上下胃肠道均受累的儿童史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症病例的成功药物治疗。