소아 스티븐슨-죤슨증후군과 독성표피괴사용해의 임상적 특성
Clinical characteristics of pediatric Stevens-Johnson syndrome and toxic epidermal necrolysis
Abstract
Objective. Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) is a very rare acute muco-cutaneous disease. There were many reports about SJS or TEN occurred in adults, but not in children. We evaluated clinical characteristics and treatment of pediatric SJS and TEN. Methods: We reviewed medical records of children with SJS or TEN in Seoul St. Mary’s hospital and Incheon St. Mary’s hospital between January, 2010 and December, 2016. We evaluated clinical findings, course, probable etiology, and treatments retrospectively. Results: Ten patients were diagnosed with SJS/TEN, and one patient had a recurrent episode, so enrolled twice. Most common symptom was skin lesion with fever. Two children progressed from SJS to SJS/TEN overlap syndrome. The probable causes were anti-epileptics (2 cases), and related with infections including mycoplasma (6 cases), EBV (1 event), and varicella zoster (1 case). Recurrent case was identified as mycoplasma and EBV infection. All patients received prednisolone or methylprednisolone. Six cases were treated with intravenous immunoglobulin (IVIG). Mean hospitalization day was longer with IVIG group than without IVIG. Conclusions: According to our study, pediatric SJS/TEN has variable manifestations from mild course controlled by oral steroid to progression severe mucocutaneous involvement. We have to consider close observation and early immunomodulation therapy to prevent progression to life-threatening course.