Sindroma Stevens Johnson
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PENYAKIT DARURAT KULIT
Dr. Rina Gustia, SpKKBag.Ilmu Kes Kulit dan KelaminFK-Unand/RS M.Jamil Padang
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Penyakit darurat kulit
Sindroma Stevens-Johnson Nekrosis Epidermal Toksik Penyakit kulit berlepuh
Pemfigus vulgaris Pemfigus foliaseus Pemfigus eritematosus Pemfigus veetans
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Sindroma Stevens-Johnson(SSJ)
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Sindroma Stevens-Johnson
• Suatu reaksi mukokutaneus akut• Mengancam jiwa• Berupa nekrosis yang ekstensif• Lepasnya epidermis kurang dari 10%• Dapat menyebabkan kematian • Terapi tepat dan cepat
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SSJ
Sinonim Sindroma mukokutanea-okular Eritema multiformis tipe Hebra Eritema bulosa maligna
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Epidemiologi
Di Bag. I Kes Kulit dan KelaminRS Dr.M.Jamil Padang (2004–2008)
Terdapat 31 kasus SSJ Tdd 19 pasien wanita, 12 pria Usia termuda 14 tahun Usia tertua pria 70 tahun Usia rata-rata 25-35 tahun
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TRIAS SSJ
KelainanKulit Selaput lendir di orifisium MataKeadaan umum bervariasi ringan sampai berat
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Trias
Kelainan kulit Eritema Vesikel – bula Erosi Purpura Kelainan generalisata
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Trias
Kel. selaput lendir di orifisium Mukosa mulut 100 % Genitalia 50 % Lubang hidung 8 % Anus 4 % Kelainan : vesikel, bula, erosi, ekskoriasi
krusta hitam tebal Pasien sukar menelan
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Trias
Kelainan mata 80 % Konjungtivitis Perdarahan Ulkus kornea Iritis Kelopak mata
Sukar di buka, lengket Sekret >> purulen
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Apa penyebab SSJ
Terutama obat
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Obat termasuk jamu
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Termasuk jamu
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Bagaimana caranya ?
Per oral Injeksi
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Bagaimana caranya
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Bagaimana caranya
Per anal
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Patogenesis
Melalui reaksi hipersensitivitas Tipe II - III
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Gambaran klinisKapan terjadi ?
Biasanya 4 sampai 30 hari setelah terpapar obat
Gejala nonspesifik sepertidemam, sakit kepala, nyeri tenggorok mialgia dan atralgia
Mata perih dan rasa terbakar Keadaan umum ringan sampai berat Berat penurunan kesadaran
soporous sampai koma
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Gambaran klinis kelainan kulit
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Kelainan mukosa mulut
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Kelainan mukosa
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Kelainan mata
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erosi
ekskoriasi
krusta hitam
kelainan mukosa genitalia
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Penyebab lain
Infeksivirus, bakteri, parasit, mikoplasma
neoplasma vaksinasi kehamilan radioterapi sebagian tidak diketahui
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Obat sebagai penyebab Lebih dari 100 jenis obat
Paling sering golongan sulfa penisilin dan derivatnya analgetik/antipiretik antikonvulsan : karbamazepin,
hidrantoin dan fenitoin antiinflamsi nonsteroid seperti
butazon dan oksikam.
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PEMERIKSAAN LABORATORIUM
Hasil tidak khas Lekositosis :infeksi bakteri Lakukan kultur darah Eosinofilia kemungkinan alergi
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DIAGNOSIS BANDING
Nekrolisis epidermal toksik (NET) NET terdapat epidermolisis. Pemfigus vulgaris tes Nikolsky positif
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Diagnosis banding
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PENATALAKSANAANUmum
• Stop semua obat tersangka• Perhatikan keadaan umum• Keseimbangan cairan dan elektrolit• Koreksi kalori• Perawatan mata • Perawatan mulut• Mencegah infeksi• Ruangan ICU suhu 28 – 30˚C
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PENATALAKSANAANKhusus
Sistemik Kortikosteroid (deksametason IV) Imunoglobulin IV Siklosporin A Plasmaferesis / hemodialisa Anti- TNF
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Cara pemberian KS
Deksametason 4-6 x 5 mg/hari Masa krisis teratasi (2-3 hr) Dosis diturunkan 5 mg/hr 5 mg Lanjutkan dengan
prednison 20 mg 10 mgLama pengobatan : 10 hari
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Tranfusi darah 300 cc 2 hari Vitamin C 500 mg- 1000 mg IV Hemostatik
Tidak ada perubahan Purpura luas Leukopeni
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Penatalaksanaan khusus
Topikal Kompres NaCl 0,9 % Kortikosteroid : Hidrokortison 2,5 %
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Pencegahan
Edukasi pasien tidak memakai obat tanpa R/ dokter
Memberikan catatan obat yang diduga jadi penyebab
Selalu A/ riwayat alergi terhadap obat Mengetahui jenis-jenis obat yang
berisiko jadi penyebab Jika timbul reaksi yang tdk diinginkan
dari obat stop segera
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KESIMPULAN
SSJKasus gawat darurat kulitDapat mengancam jiwaDengan penatalaksanaan yang cepat dan tepat prognosis baik
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NEKROLISIS EPIDERMAL TOKSIK (NET)
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Definisi Penyakit berat Klinis berupa epidermolisis generalisata Dapat mengenai selaput lendir di
orifisium dan mata
Sinonim Sindrom Lyell
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Etiologi
Sama dengan SSJ - alergi obat - infeksi - vaksinasi - penyakit graft versus host - neoplasma - radiasi
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Patogenesis
Sama dengan SSJ reaksi hipersensitif tipe II (sitolitik)
sel targetnya epidermis epidermollisis
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Gejala klinis Penyakit berat dan akut Gejala prodromal Eritema generalisata vesikel +bula
purpura Epidermolisis Nikolsky sign (+) Dapat mengenai selaput lendir erosi,
ekskoriasi, krusta kehitaman Dapat disertai kelainan pd mata
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Komplikasi
Ketidaksimbangan cairan & elektrolit
Kelainan ginjal nekrosis tubular akut, glomerulonefritis
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Histopatologi
- Vakuolisasi dan nekrosis sel basal sepanjang perbatasan dermal-epidermal
- Sel radang didermis hanya sedikit terdiri atas limfohistiosit
- Pada lesi lanjut nekrosis eosinofilik sel epidermis dg pembentukan bula subepidermal.
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Diagnosis banding SSJ epidermolisis (-), KU tidak seburuk NET Staphylococcus Scalded Skin Syndrome
[SSSS] klinis mirip NET terdpt epidermolisis tetapi selaput lendir
tidak dikenaisering pada anak-anak < 5 tahundisebabkan stafilokokus aureus
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PengobatanMirip 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
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Topikal Sulfadiazin perak Mulut betadine gargle Bibir kenalog in ora base
Prognosis Baik jika disebabkan infeksi Kelainan
kulit luas 50-70% prognosisnya buruk. Buruk, jika luas 50 – 70 % Angka kematian 16%
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PEMFIGUS
• Kumpulan penyakit kulit autoimun• Berbula kronik• Menyerang kulit dan membran mukosa• Histologik :Bula intraepidermal akantolisis• Imunopatologik : ditemukan antibodi
terhadap komponen desmosom.
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Bentuk1. Pemfigus vulgaris2. Pemfigus vegetans3. Pemfigus foliaceus4. Pemfigus eritematosus
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Penyebab: Termasuk penyakit auto-imun auto-
antibodi terhadap jaringan interseluler epidermis
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Gambaran klinis
- Keadaan umum : biasanya buruk- Mengenai semua selaput lendir dgn epitel skuamosa - Stomatitis aftosa terjadi pada banyak penderita sebelum diagnosis pasti ditegakkan
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Gambaran klinis
Bersifat kronis residif Bula generalisata/ seluruh tubuh Bula
lembek, cairan jernih seropurulen bahkan hemoragik.
mudah pecah meninggalkan hiperpigmentasi tanpa sikatriks
Tanda Nikolsky (+)
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Diagnosis dan Diagnosis banding: D/ berdasarkan gejala klinis khas yaitu
sifat bula yg lembek dan tanda Nikolsky positif
Pemeriksaan penunjang : Histopatologi/Tzank test
Imunofluoresen, ditemukan Ig G pada interseluler epidermis
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PENGOBATAN.Sistemik
Kortikosteroid : prednison 80-120 mg- Dosis tinggi dan jangka panjang Antibiotika utk infeksi sekunder Anabolik Diet TKTPTopikal Kompres PK 1/10.000, krim kortikosteroid
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Diagnosa banding.
- Dermatitis herpetiformis Duhring.- Pemfigoid bulosa.- Sindroma Steven Johnson- Impetigo vesiko bulosa.- NET
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Gambar-Gambar
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Gambar
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Gambar
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TERIMAKASIH