고용량 면역글로불린 정맥주사로 치료한 2세 남아의 독성표피괴사용해 1례

Abstract

Toxic epidermal necrolysis(TEN) is a severe drug induced life-threatening disease and an acute illness. This disease is characterized by rapid onset of widespread necrosis resulting in sloughing of epidermis. The incidence of TEN is very rare, with approximately 0.5 to 1.4 cases per million per year. but TEN has a high mortality rate of 25-40 percent. Therapy for TEN is primarily aimed at supportive care. Treatment with systemic corticosteroid, immunosuppresive agent such as cyclosporine, cyclophosphamide, pentoxifyllin or plasmapheresis have not been shown to improve outcome. Recently, administration of high dose intravenous immunoglobulin(IVIG) has been shown to result in rapid improvement in patients with TEN. There have been several reports of the benefit of IVIG in adult patients with TEN. However we could not find using IVIG in pediatric patient with TEN in Korea. We have experienced improvement in a 2 years old boy with TEN after using high dose IVIG.