Oral Cyclosporine Treatment for Four Pediatric Patients With Toxic Epidermal Necrolysis That Showed No Response to High-dose Corticosteroids in Combination With Intravenous Immunoglobulin: A Case Series

IF 1.6 Q3 MEDICINE, RESEARCH & EXPERIMENTAL Current Therapeutic Research-clinical and Experimental Pub Date : 2025-01-01 DOI:10.1016/j.curtheres.2024.100767

Peijing Li MD , Qin Yao MD , Yuanyuan Wang MD , Xipeng Xu MD

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Abstract

Background

Immunosuppressive agents like cyclosporine have proven effective in some pediatric cases, although there are limited case reports considering potential risks such as secondary infections.

Objective

This study investigated the safety and efficacy of Cyclosporine A in children who did not respond to high-dose corticosteroids combined with intravenous immunoglobulin (IVIG).

Methods

We reported four pediatric patients diagnosed with toxic epidermal necrolysis (TEN) received treatment at our institution. All patients were previously healthy children with a median age of 7 years, comprising three boys and one girl (Table 1). Epidermal exfoliation and vesicular lesions ranged from 32.5% to 54.5% of the body surface area (BSA). Despite the administration of treatment comprising high-dose corticosteroids and intravenous immunoglobulin (IVIG), new cutaneous herpes continually emerged. This prompted a transition to cyclosporine treatment (3–5 mg/kg/d) administered in 1–2 oral doses.

Results

Lesions stopped progressing, and bullous lesions started epithelialization after 13–27 days of hospitalization. Cases 1 and 2 faced secondary bacterial and fungal infections, respectively, and their temperatures stabilized after administration of antibiotics. Cases 3 and 4 experienced fever again when the dosage of corticosteroids was tapered off, with no discernible evidence of infection. The patients’ temperatures normalized upon the continuation of cyclosporine therapy. Among the patients, three presented asymptomatic elevated serum amylase, one of which met the diagnostic criteria for acute pancreatitis. Two children showed mildly raised aminotransferases, with one experiencing mild coronary artery dilation, two contracted onychomadesis, and three developed corneal ulceration/keratitis and atretoblepharia, which eventually resolved after vigorous ophthalmologic treatment. None of the children had any permanent sequelae after being discharged from the hospital for six months.

Conclusions

Cyclosporine A is generally safe and effective for children who fail to respond to high-dose corticosteroids in combination with IVIG.

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口服环孢素治疗4例对大剂量皮质类固醇联合静脉注射免疫球蛋白无反应的中毒性表皮坏死松解患儿：一个病例系列

背景：免疫抑制剂如环孢素已被证明对一些儿科病例有效，尽管考虑到继发感染等潜在风险的病例报告有限。目的：本研究探讨环孢素A对大剂量皮质类固醇联合静脉注射免疫球蛋白（IVIG）无反应的儿童的安全性和有效性。方法：我们报告了4例诊断为中毒性表皮坏死松解症（TEN）的儿科患者在我院接受治疗。所有患者均为健康儿童，中位年龄为7岁，包括3名男孩和1名女孩（表1）。表皮脱落和水疱病变占体表面积（BSA）的32.5%至54.5%。尽管给予高剂量皮质类固醇和静脉注射免疫球蛋白（IVIG）治疗，新的皮肤疱疹仍不断出现。这促使改用环孢素治疗（3-5 mg/kg/d），分1-2次口服。结果：13 ~ 27 d后病变停止进展，大疱性病变开始上皮化。病例1和2分别面临继发性细菌和真菌感染，并在给予抗生素后体温稳定。病例3和4在皮质类固醇剂量逐渐减少时再次出现发热，没有明显的感染证据。继续环孢素治疗后患者体温恢复正常。3例患者血清淀粉酶无症状升高，其中1例符合急性胰腺炎诊断标准。2例患儿转氨酶轻度升高，1例发生轻度冠状动脉扩张，2例发生甲发育，3例发生角膜溃疡/角膜炎和眼膜粘连，经大力眼科治疗后最终消退。出院6个月后，所有患儿均无永久性后遗症。结论：环孢素A对于大剂量皮质类固醇联合IVIG治疗无效的儿童通常是安全有效的。

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来源期刊

Current Therapeutic Research-clinical and Experimental 医学-药学

CiteScore

3.50

自引率

0.00%

发文量

审稿时长

3 months

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