Yamashita Hiroyuki, Ueda Yo, Takahashi Yuko, Mimori Akio
Division of Rheumatology, National Center for Global and Health Medicine.
Kansenshogaku Zasshi. 2012 Jul;86(4):419-24. doi: 10.11150/kansenshogakuzasshi.86.419.
A 58-year-old female with a history of dermatomyositis was receiving large oral doses of steroids. She had pulmonary tuberculosis and developed a fever, systemic exudative erythema, exanthema, and epidermolysis covering 30% of her body surface area while being treated with four agents, including isoniazid (INH) and rifampicin (RFP). Histopathologically, eosinophilic necrosis was observed in all layers of the epidermis and a diagnosis of Stevens-Johnson syndrome (SJS) progressive toxic epidermal necrolysis (TEN) was made. The drugs suspected in the drug-induced lymphocyte stimulation test (DLST) re-testing were INH and RFP, and the DLST was considered to be important during the recovery period as well as in the acute phase. Early treatment with plasma exchange therapy and large quantities of intravenous immunoglobulin (IVIG) was successful. Plasma exchange therapy and IVIG are extremely effective when SJS and TEN occur in a patient already on high-dose steroid therapy. Note that the incidence of SJS and TEN is believed to be higher in patients with collagen disease, such as in our case, as compared to the general population.
一名58岁有皮肌炎病史的女性正在接受大剂量口服类固醇治疗。她患有肺结核,在接受包括异烟肼(INH)和利福平(RFP)在内的四种药物治疗时,出现发热、全身性渗出性红斑、皮疹,且表皮松解面积覆盖体表的30%。组织病理学检查显示,表皮各层均观察到嗜酸性坏死,诊断为史蒂文斯 - 约翰逊综合征(SJS)进展性中毒性表皮坏死松解症(TEN)。药物诱导淋巴细胞刺激试验(DLST)复查中怀疑的药物为INH和RFP,DLST在恢复期以及急性期都被认为很重要。早期采用血浆置换疗法和大量静脉注射免疫球蛋白(IVIG)治疗取得成功。当SJS和TEN发生在已经接受大剂量类固醇治疗的患者身上时,血浆置换疗法和IVIG极为有效。请注意,与普通人群相比,如我们病例中的胶原病患者发生SJS和TEN的发生率据信更高。