Toksik epidermal nekroliz tedavisinde siklosporin a kullanımı; Olgu sunumu ve literatür derlemesi
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Date
2014-06-16
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Uludağ Üniversitesi
Abstract
Toksik epidermal nekroliz (TEN) sıklıkla ilaçlar tarafından tetiklenen, deri, göz, mukozalar ve birden çok organı etkileyebilen, ciddi ve hayatı tehdit eden akut mukokütanöz bir hastalıktır. On bir yaşında kız hasta, epilepsi nedeni ile 3 yıldır valproik asit kullanırken tedavisine bir ay önce lamotrigin eklenmişti. Hasta iki gün önce başlayan ateş, halsizlik, ağız içinde yaralar, gözlerde kızarıklık, sulanma, deride döküntü ve çok sayıda büllöz lezyonlar ile başvurdu. Fizik muayenede, genel durumu kötü, tüm vücutta yaygın makülopapüler döküntüler, purpurik maküller, tipik olmayan hedef benzeri deri lezyonları, vücut yüzeyinin %30’dan fazlasını etkileyen değişik evrelerde en büyüğü 6-10 cm çaplarında çok sayıda büller saptandı. Ağız mukozasında ülsere lezyonlar, her iki gözde keratit, blefarit ve konjunktival hiperemi vardı. Hastaya mevcut bulguları ile TEN tanısı konuldu. Lamotrigin tedavisi kesildi, destek tedavisi, metilprednisolon, intravenöz immunglobulin, deri ve göz lezyonlarına yönelik bakım ve gerekli topikal tedaviler uygulandı. İzleminde klinik tablo ve deri lezyonlarında iyileşme olmaması üzerine hastanın tedavisine siklosporin A eklendi. Siklosporin A tedavisine iyi cevap alınan olgunun klinik tablo ve deri lezyonlarında belirgin iyileşme gözlendi. TEN tedavisinin esasını şüpheli ilacın kesilmesi ve destek tedavisi oluşturmaktadır. TEN tedavisinde, sistemik kortikosteroidler, İVİG gibi immünsüpresif ajanlar tedavide kullanılabilmektedir. Tedaviye dirençli veya ciddi TEN olgularında siklosporin kullanımı tedaviye olumlu katkı sağlayabilir. Siklosporinin TEN tedavisindeki yeri ve etkilerini araştırmaya yönelik ayrıntılı çalışmalara ihtiyaç vardır.
Toxic epidermal necrolysis (TEN) is a serious and life-threatening acute mucocutaneous disease that is often triggered by drugs, can affect skin, eyes, mucous membranes and multiple organs. While an eleven-year-old female patient with epilepsy had been using valproic acid for three years, lamotrigine was added to the treatment one month ago. The patient was admitted complaints such as fever, malaise, sores in the mouth, eye redness, watering of eyes, rashes and many bullous lesions in the skin that had started just 2 days earlier. On the physical examination, whose overall situation was bad, that were common maculopapular rash, purpuric macules on the whole body, atypical target-like skin lesions, and many bullae that affected more than 30% of the body in different stages and whose largest one was 6-10 cm in diameter were detected. There were ulcerated lesions in the oral mucosa and keratitis, blepharitis and conjunctival hyperemia in both eyes. The patient was diagnosed with TEN through the existing findings. The lamotrigine treatment was discontinued; the supportive treatment, methylprednisolone, intravenous immunoglobulin, necessary care for skin and eye lesions and the necessary topical treatment were applied. As no improvement was observed in clinical picture and skin lesions in the follow-up period, cyclosporine A was added to the treatment. Significant improvement was observed in the clinical picture and skin lesions of the patient who responded well to the treatment of cyclosporine A. The basis of the treatment of TEN is composed of discontinuation of the suspicious drug and the supportive therapy. The immunosuppressive agents such as systemic corticosteroids and IVIG therapy may be used in the treatment of TEN. Cyclosporine can positively contribute to the treatment in cases who are resistant to treatment and with a severe TEN condition. There is a need for in-depth studies to investigate the place and impact of cyclosporine in the treatment of TEN.
Toxic epidermal necrolysis (TEN) is a serious and life-threatening acute mucocutaneous disease that is often triggered by drugs, can affect skin, eyes, mucous membranes and multiple organs. While an eleven-year-old female patient with epilepsy had been using valproic acid for three years, lamotrigine was added to the treatment one month ago. The patient was admitted complaints such as fever, malaise, sores in the mouth, eye redness, watering of eyes, rashes and many bullous lesions in the skin that had started just 2 days earlier. On the physical examination, whose overall situation was bad, that were common maculopapular rash, purpuric macules on the whole body, atypical target-like skin lesions, and many bullae that affected more than 30% of the body in different stages and whose largest one was 6-10 cm in diameter were detected. There were ulcerated lesions in the oral mucosa and keratitis, blepharitis and conjunctival hyperemia in both eyes. The patient was diagnosed with TEN through the existing findings. The lamotrigine treatment was discontinued; the supportive treatment, methylprednisolone, intravenous immunoglobulin, necessary care for skin and eye lesions and the necessary topical treatment were applied. As no improvement was observed in clinical picture and skin lesions in the follow-up period, cyclosporine A was added to the treatment. Significant improvement was observed in the clinical picture and skin lesions of the patient who responded well to the treatment of cyclosporine A. The basis of the treatment of TEN is composed of discontinuation of the suspicious drug and the supportive therapy. The immunosuppressive agents such as systemic corticosteroids and IVIG therapy may be used in the treatment of TEN. Cyclosporine can positively contribute to the treatment in cases who are resistant to treatment and with a severe TEN condition. There is a need for in-depth studies to investigate the place and impact of cyclosporine in the treatment of TEN.
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Keywords
Çocuk, Toksik epidermal nekroliz, Lamotrigin, Siklosporin A, Child, Toxic epidermal necrolysis, Lamotrigine, Cyclosporine A
Citation
Canıtez, Y. vd. (2014). "Toksik epidermal nekroliz tedavisinde siklosporin a kullanımı; Olgu sunumu ve literatür derlemesi". Güncel Pediatri, 12(2), 116-122.