14例泛发性大疱性固定性药疹临床分析

Clinical analysis of 14 cases of generalized bullous fixed drug eruption

目的:比较泛发性大疱性固定性药疹(GBFDE)与中毒性表皮坏死松解症(TEN)病例的临床表现、治疗方案及疾病转归情况,探索其临床特征。方法:对我科2008-2018年收住入院的14例GBFDE患者以及中毒性表皮坏死松解症病情严重程度评分(SCORTEN)相同、皮肤剥脱面积占体表面积百分比(total body surface area, TBSA)相近的TEN患者按照1∶3的数量比进行病例对照分析。结果:GBFDE与TEN常见的致敏药物主要有抗生素、抗癫痫药、非甾体类抗炎药,GBFDE患者在是否黏膜受累、≥2处黏膜受累、住院时间等方面均显著低于TEN患者(P分别为0.002,0.000,0.000)。GBFDE患者的糖皮质激素最大使用剂量、激素开始减量时间、减量前激素总量分别为0.9±0.3mg/kg·d、5.5(4.25,7)d、4.7(3.9,5.2)mg/kg,均少于TEN患者的1.4±0.3mg/kg·d、10(7,12)d、11.9(9.3,16.7)mg/kg·d(P均=0.000);治疗过程中3例GBFDE患者(21.4%)联合应用静脉输注免疫球蛋白(intravenous immunoglobulin, IVIG),少于TEN患者(37/52,88.1%,P=0.000)。电解质紊乱是GBFDE与TEN最常见的并发症,GBFDE患者电解质紊乱、药物性肝损伤、继发感染、消化道出血等并发症的发生率均明显低于TEN患者(P分别为0.000、0.047、0.004、0.026)。结论:尽管临床表现相似,但GBFDE临床转归较TEN好,需要的糖皮质激素量更小,因而糖皮质激素相关并发症的发生率较TEN低,临床上应注意避免过度治疗。

Objective: To compare the clinical manifestations, treatments and prognosis of generalized bullous fixed drug eruption(GBFDE) and toxic epidermal necrolysis(TEN) cases with the same SCORTEN score and TBSA, and to explore the differences of their clinical characteristics and outcomes. Methods: Case-control study was performed. All the patients, including14 GBFDE patients and 42 TEN patients who had the similar SCORTEN score and TBSA, were admitted to the department of dermatology, Huashan hospital affiliated to fudan university from 2008 to 2018. Results: The common causative drugs of GBFDE and TEN in our study were mainly antibiotics, anticonvulsants, and nonsteroidal anti-inflammation drugs. The rate of mucosal involvement, ≥2 mucosal involvement, and length of hospital stay in GBFDE patients were significantly lower than that in TEN patients(P values were 0.002, 0.000, 0.000). The maximum dose of glucocorticoid used in treating GBFDE patients, the start time of dose reduction, and the total amount of glucocorticoid before dose reduction were(0.9±0.3) mg/kg·d, 5.5(4.25,7) d and 4.7(3.9, 5.2) mg/kg·d respectively, which were all significantly less than those of TEN patients[(1.4±0.3) mg/kg·d, 10(7,12)d and 11.9(9.3,16.7) mg/kg·d](all P=0.000). The numbers of GBFDE patients(3/14, 21.4%) received IVIG were significantly less than that of TEN patients(37/52, 88.1%, P=0.000). The most common complication of GBFDE and TEN was electrolyte disturbance. The incidence of electrolyte disorder, drug-induced liver injury, secondary infection and gastrointestinal bleeding in GBFDE patients were all significantly lower than that of TEN patients(P=0.000, 0.047, 0.004 and 0.026 respectively). Conclusion: Despite the similar clinical manifestations, GBFDE has a better clinical outcome than TEN and requires less glucocorticoids treatment. Therefore, the incidence of glucocorticoid-related complications in GBFDE patients is lower than that of TEN patients. The risk of overtreatment should be considered and avoided.