Pannu Bibek S, Egan Ashley M, Iyer Vivek N
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Division of General Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Respir Med Case Rep. 2016 Jan 20;17:54-6. doi: 10.1016/j.rmcr.2016.01.006. eCollection 2016.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare but serious idiosyncratic drug reactions characterized by diffuse muco-epidermoid injury and high mortality. Keratinocytes in both skin and mucous membranes (including eyes, mouth and genitalia) are injured resulting in a diffuse maculopapular rash, blistering lesions and epithelial detachment with minimal force (Nikolsky's sign). SJS is typically diagnosed when less than 10% of the skin surface is involved and the term TEN is used in cases with more than 30% involvement. Respiratory involvement in SJS-TEN is common with 30-50% of cases demonstrating respiratory epithelial sloughing with severe short and long term complications. Patients who survive SJS-TEN are often left with impaired respiratory function and bronchiolitis obliterans. Cases of bronchiolitis obliterans with SJS/TEN have been very rarely reported. We report a case of phenytoin induced SJS/TEN followed by severe bronchiolitis obliterans in an adult patient. The presentation, pathophysiology and management of SJS/TEN related bronchiolitis obliterans is also reviewed.
史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)均为罕见但严重的特异质性药物反应,其特征为弥漫性黏膜表皮损伤且死亡率高。皮肤和黏膜(包括眼睛、口腔和生殖器)中的角质形成细胞受损,导致弥漫性斑丘疹、水疱性损害以及轻微外力即可引起上皮剥脱(尼氏征)。当皮肤受累面积小于10%时通常诊断为SJS,而受累面积超过30%的病例则诊断为TEN。SJS-TEN常累及呼吸系统,30%-50%的病例出现呼吸道上皮脱落,并伴有严重的短期和长期并发症。SJS-TEN存活患者常遗留呼吸功能受损和闭塞性细支气管炎。SJS/TEN合并闭塞性细支气管炎的病例报道极少。我们报告1例成年患者因苯妥英钠诱发SJS/TEN,随后发生严重闭塞性细支气管炎的病例。本文还对SJS/TEN相关闭塞性细支气管炎的临床表现、病理生理学及治疗进行了综述。