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

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...

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Main Authors: Peijing Li, MD, Qin Yao, MD, Yuanyuan Wang, MD, Xipeng Xu, MD
Format: Article
Language:English
Published: Elsevier 2025-01-01
Series:Current Therapeutic Research
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Online Access:http://www.sciencedirect.com/science/article/pii/S0011393X24000377
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author Peijing Li, MD
Qin Yao, MD
Yuanyuan Wang, MD
Xipeng Xu, MD
author_facet Peijing Li, MD
Qin Yao, MD
Yuanyuan Wang, MD
Xipeng Xu, MD
author_sort Peijing Li, MD
collection DOAJ
description 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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spelling doaj-art-2750b030bfd84563bc145f4a869b692f2024-12-23T04:18:56ZengElsevierCurrent Therapeutic Research0011-393X2025-01-01102100767Oral 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 SeriesPeijing Li, MD0Qin Yao, MD1Yuanyuan Wang, MD2Xipeng Xu, MD3Department of Infectious Diseases, Qingdao Women and Children's Hospital, Qingdao, ChinaDepartment of Infectious Diseases, Qingdao Women and Children's Hospital, Qingdao, ChinaDepartment of Infectious Diseases, Qingdao Women and Children's Hospital, Qingdao, ChinaDepartment of Critical Medicine, The Affiliated Hospital of Qingdao University, Qingdao, China; Address correspondence to: Xipeng Xu, Department of Critical Medicine, The Affiliated Hospital of Qingdao University, No.16 Jiangsu Road, Qingdao 276700, China.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.http://www.sciencedirect.com/science/article/pii/S0011393X24000377CyclosporinPediatricToxic epidermal necrolysisTreatment
spellingShingle Peijing Li, MD
Qin Yao, MD
Yuanyuan Wang, MD
Xipeng Xu, MD
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
Current Therapeutic Research
Cyclosporin
Pediatric
Toxic epidermal necrolysis
Treatment
title 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_short 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
title_sort 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
topic Cyclosporin
Pediatric
Toxic epidermal necrolysis
Treatment
url http://www.sciencedirect.com/science/article/pii/S0011393X24000377
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