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논문분류	춘계학술대회 초록집
제목	Two cases of toxic epidermal necrolysis associated with deflazacort therapy in nephrotic syndrome were successfully treated with cyclosporine A
저자	Myung-Hee Lim*, Sun-Young Park, Young-Hoon Park
출판정보	2016; 2016(1):
키워드	Cyclosporine, Deflazacort, Nephrotic syndrome, Toxic epidermal necrolysis
초록	ackground: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, mucocutaneous diseases that can be life-threatening. They are thought to be caused by a severe adverse reaction to drugs including deflazacort that is usually used in patients with nephrotic syndrome because of the fewer side effects (such as growth retardation, osteoporosis, Cushing syndrome, etc.) than predinosolone. There is currently no gold standard treatment established for SJS/TEN, particularly in children. Two therapies of SJS/TEN in children, intravenous immunoglobulin (IVIG) and corticosteroid pulses, are widely accepted, although controversy exists. The second-line drugs are immunosuppressant including cyclosporine A (CSA). In most cases, IVIG therapy were reported to be effective in TEN that is associated with deflazacort therapy in nephrotic syndrome.1,2 In this case report, however, we used CSA and successfully treated two patients with nephrotic syndrome who suffered from TEN during deflazacort medication. Methods: This report describes two cases of TEN in a 12-year-old boy and a girl who presented with nephrotic syndrome that developed during treatment with deflazacort. Each of them was admitted with skin lesions consisting of multiple erythematous maculae and mucosal involvement of the lips, face, and both extremities. It occurred during the 5 weeks of deflazacort (72 mg/day) administration for the boy and during the 2 weeks of tapering medication dose ( 24 mg/day) after the 5 weeks of deflazacort (72 mg/day) for the girl. Within 3 days, both developed widespread skin lesions more than 30 percents of the body surface. Results: Corticosteroid was changed from deflazacort to prednisolone, and CSA (3 mg/kg/day, daily maximum 150mg) was administered. From 1 week after using CSA, the patients showed rapid improvement in symptoms and were subsequently discharged 2 weeks after admission to the hospital without any complication, and then prednisolone was tapered and discontinued. Conclusion: We introduced the treatment with CSA in TEN associated with deflazacort in nephritic syndrome, instead of IVIG or corticosteroids. SJS/TEN are associated with altered immunological conditions. The detachment of the epidermis in TEN is known to be caused by the necrosis of keratinocytes following apoptosis. The exposure to a drug induces maturation of the CD 8+ T lymphocytes, responsible for apoptosis, into cytotoxic T lymphocytes. CSA inhibits those immune responses and interferes to cell apoptosis which progresses to TEN. CSA (3 to 5 mg/kg/day) associated with a rapid re-epithelialization rate has been shown to decrease the duration of active disease. Most of all, CSA also used as a treatment for nephritic syndrome. In terms of infection risk, CSA is more beneficial than corticosteroid pulse therapy which may increase the possibility of infection as they suppress immunity. Moreover, the use of CSA may offer cost effective treatment than IVIG in Korea.
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