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静脉注射免疫球蛋白治疗中毒性表皮坏死松解症:三例系列报道

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin: a series of three cases.

作者信息

Comparin Cristiane, Hans Filho Günter, Takita Luiz Carlos, Costa Nayara de Castro Wiziack, Nascimento Roberta Ayres Ferreira do, Nanni Lidiane de Oliveira Costa

机构信息

Department of Dermatology, Teaching Hospital, Federal University of Mato Grosso do Sul, Campo Grande, MS, Brazil.

出版信息

An Bras Dermatol. 2012 May-Jun;87(3):477-81. doi: 10.1590/s0365-05962012000300022.

DOI:10.1590/s0365-05962012000300022
PMID:22714769
Abstract

Stevens-Johnson's syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatoses, that lead to keratinocyte apoptosis induced by interactions between Fas (cell death receptor) and soluble Fas-ligand, present in serum of Stevens-Johnson's syndrome / toxic epidermal necrolysis patients. Anti-Fas antibodies in intravenous immunoglobulin (IVIG) would block the apoptosis cascade. Three cases of toxic epidermal necrolysis occurred in one male and two female patients, after use of allopurinol, leprosy multidrug therapy concomitant with dipyrone, and diclofenac. The cases were treated with intravenous immunoglobulin 2-3 mg/kg and prednisone 20-50 mg/day. The interruption of new lesions outbreak and reepithelization were extremely fast after the use of intravenous immunoglobulin, without adverse effects. Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising.

摘要

史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是危及生命的皮肤病,由Fas(细胞死亡受体)与史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症患者血清中存在的可溶性Fas配体之间的相互作用诱导角质形成细胞凋亡。静脉注射免疫球蛋白(IVIG)中的抗Fas抗体可阻断凋亡级联反应。一名男性和两名女性患者在使用别嘌醇、麻风病联合化疗并伴有双氯芬酸以及双氯芬酸后发生了三例中毒性表皮坏死松解症。这些病例接受了2 - 3mg/kg的静脉注射免疫球蛋白和20 - 50mg/天的泼尼松治疗。使用静脉注射免疫球蛋白后,新皮疹爆发的中断和上皮再形成极为迅速,且无不良反应。需要进行对照研究以证实静脉注射免疫球蛋白在史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症中的疗效,但结果似乎很有前景。

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