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Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens–Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN
Pediatric Drugs ( IF 3.4 ) Pub Date : 2022-06-09 , DOI: 10.1007/s40272-022-00515-0
Michele L Ramien 1, 2 , Danny Mansour 3 , Neil H Shear 4
Affiliation  

Pediatric Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threating blistering diseases triggered by medications that affect the skin and mucosae. Drug-induced epidermal necrolysis is a better term for medication-triggered cases because there is a spectrum of disease severity that otherwise is divided into the separate entities of SJS, overlap SJS/TEN, and TEN. This manuscript reviews the management of drug-induced epidermal necrolysis (DEN), including diagnosis, investigations to exclude differential diagnoses, and treatment. Diagnosis of DEN relies on clinical features and a detailed medication history. The primary differential diagnosis is reactive infectious mucocutaneous eruption, which can be clinically distinguished by its disproportionate mucous membrane involvement relative to (sparse or absent) skin lesions. Identification and discontinuation of culprit medications is the mainstay of treatment of DEN. Early initiation of immunomodulatory therapy may prevent progression, reducing maximal disease severity and the risk of sequelae. A checklist approach to detailed management of DEN is proposed.



中文翻译:

儿科患者药物性表皮坏死松解症 (DEN) 的管理:从药物性 Stevens-Johnson 综合征、重叠和中毒性表皮坏死松解症转变为 DEN 的单一统一诊断

小儿史蒂文斯-约翰逊综合征 (SJS) 和中毒性表皮坏死松解症 (TEN) 是罕见但危及生命的水疱疾病,由影响皮肤和粘膜的药物引发。药物引起的表皮坏死松解症是药物引发病例的更好术语,因为有一系列疾病严重程度,否则分为 SJS、重叠 SJS/TEN 和 TEN 的单独实体。本手稿回顾了药物性表皮坏死松解症 (DEN) 的管理,包括诊断、排除鉴别诊断的调查和治疗。DEN 的诊断依赖于临床特征和详细的用药史。主要鉴别诊断是反应性感染性皮疹,临床上可以通过其与(稀疏或不存在)皮肤病变的不成比例的粘膜受累来区分。识别和停用罪魁祸首药物是治疗 DEN 的中流砥柱。早期开始免疫调节治疗可以防止疾病进展,降低最大的疾病严重程度和后遗症的风险。提出了一种详细管理 DEN 的清单方法。

更新日期:2022-06-09
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