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NEKROLISIS EPIDERMAL TOKSIK (NET)

NEKROLISIS EPIDERMAL TOKSIK (NET). Definisi. Penyakit berat Klinis berupa epidermol i sis generalisata Dapat mengenai selaput lendir di orifisium dan mata Sinonim Sindrom Lyell. Etiologi. Sama dengan SSJ - alergi obat - infeksi - vaksinasi - penyakit graft versus host

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NEKROLISIS EPIDERMAL TOKSIK (NET)

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  1. NEKROLISIS EPIDERMAL TOKSIK (NET)

  2. Definisi • Penyakitberat • Klinisberupaepidermolisis generalisata • Dapatmengenaiselaputlendirdiorifisiumdanmata Sinonim • Sindrom Lyell

  3. Etiologi • Sama dengan SSJ - alergi obat - infeksi - vaksinasi - penyakit graft versus host - neoplasma - radiasi

  4. Patogenesis • Samadengan SSJ  reaksihipersensitiftipe II (sitolitik) seltargetnya epidermis  epidermollisis

  5. Gejala klinis • Penyakitberatdanakut • Gejalaprodromal • Eritemageneralisata vesikel +bulla purpura • Epidermolisis nikolsky sign (+) • Dapatmengenaiselaputlendir  erosi, ekskoriasi, krustakehitaman • Dapatdisertaikelainan pd mata

  6. Komplikasi • Ketidaksimbangan cairan & elektrolit • Kelainan ginjal  nekrosis tubular akut + glomerulonefritis Histopatologi vakuolisasi dan nekrosis sel basal sepanjang perbatasan dermal-epidermal. Sel radang didermis hanya sedikit terdiri atas limfohistiosit. Pada lesi lanjut terdapat nekrosis eosinofilik sel epidermis dg pembentukan bula subepidermal.

  7. Diagnosis banding • SSJ, perbedaan SSJ epidermolisis (-), KU tidakseburuk NET • Staphylococcus Scalded SkinSyndrome [SSSS] klinismirip NET, terdptepidermolisistetapiselaputlendirtidakdikenai. Penyebab SSSS ialah Staphylococcus aureus, biasanya pd anakdibawah 5 th. Gambaranhistopatologikjugaberbeda.

  8. Pengobatan Mirip SSJ : • Obat tersangka  stop • IVFD  D5%:NaCl 0,9%:Rl  1:1:1 / 8 jam • Kortikosteroid dosis tinggi (deksametason 40mg IV/hr  ada perbaikan tapp off) • Antibiotik • Bila K darah ↓  KCl 3 x 500mg • Perbaikan (-)  transfusi darah 300cc selama 2 hr • Purpura luas  vit C 500-1000 mg/hr • Diet rendah garam tinggi protein

  9. Topikal • Sulfadiazinperak • Mulut betadine gargle • Bibir kenalog in ora base Prognosis • Disebabkaninfeksi prognosis lebihbaikdaripadaalergiobat. Kelainankulitluas 50-70% prognosisnyaburuk. • Angkakematian 16%

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