KEGAWATDARUTAN KULITDr.M.Syafei Hamzah,SpKK

1. STEVEN JOHNSON SYNDROME

2. TOKSIK EPIDERMOLISIS NEKROTIKAN

STEVEN JOHNSON SYNDROMDEFINISI : = kumpulan gejala penyakit pada kulit dan mukosa disertai gejala sistemik, -> Eritema multiforme berat ; bersifat akut, ditandai trias -> kulit, mukosa, mata.

Epidemiologi : - insiden 1,2 6 per sejuta tiap th - tidak ada perbedaan ras - semua umur

Etiologi : -> pasti belum diketahui.1. Alergi obat >> a.l. sulfa, penisilin,NSAID, antikonvulsi antipiretik, jamu. 2. Infeksi -> virus, bakteri, parasit - neoplasma, vaksinasi, radioterapi

Patogenesis :Pasti -> tidak diketahui reaksi hipersensitivitas tipe III dan IV - R tipe III -> tbtk komplek Ag-Ab -> mikropresipitasi -> aktivasi sistem komplemen -> akumulasi neutrofil -> lisozim -> kerusakan jar. pd target org - R tipe IV ->limfosit T tersensitisasi kontak dg Ag yg sama -> limfokin -> R radang

Gejala Klinis :KU bervariasi : ringan-berat -> koma G/ prodromal : demam tinggi, malaise, nyeri kepala,sakit tenggorok Kelainan ->Trias SJS : kulit, mukosa,mata Kulit : eritema, vesikel,bula, -> erosi luas purpura. berat -> generalisata

Kelainan mukosa : -> mulut , faring, alat genital - vesikel, bula -> erosi , eksoriasi -> krusta kehitamanKelainan mata : -> konjuctivitis kataralis >> konjungtivitis purulen, perdarahan, simblefaron , ulkus kornea, iritis

Pengobatan :Tergantung berat ringan .Rawat, Keadaan umum, cepat life saving -> kortikosteroid - deksametasone : 4-6 x 5 mg/hari -> tappering off segera. - Antibiotika - intake cairan/elektrolit

Komplikasi :BronkopneumoniSyokKelainan ginjal -> ATNKebutaan

Prognosis :Bertindak cepat dan tepat->prognosis baik

Kematian -> 5 - 15 %

November 2007, : > Stevens Johnson Syndrome in Bandar...< Previous| Next >ARTICLE LINKS: PDF (150K)|View full-size inline imagesWorld Allergy Organization Journal:Volume WAC 2007 AbstractsNovember 2007p S276

Stevens Johnson Syndrome in Bandar Lampung, Indonesia[Abstracts: Abstracts of the XX World Allergy Congress 2007 December 2-6, 2007, Bangkok, Thailand: POSTER GROUP 3 - WEDNESDAY: DRUG ALLERGY: 863]Hamzah, M. SyafeiDr. Abdul Moeloek Hospital, Dermatovenereology Department, Bandar Lampung, Indonesia.

Background:Stevens Johnson Syndrome (SJS) is severe form of erythema multiforme with related mucocutaneus disorder, often with severe constitutional symptoms and associated high rate of mortaliy and morbidity.

Objective:To find out the incidence, etiology, treatment, lenght of stay and complication.

Methods:A 3 years retrosprctive study (January 1, 2004 through December 31, 2006) of patients admitted to dermatology department of Dr. Abdul Moeloek Hospital Lampung.

Results:Among 24 cases reviewed there were 11 (45,8%) male and 13 (54,2%) female. The youngest patient was a 10 years old girl and the oldest one a 54 years old female. We assume that the caused were antibiotic (penicillin derivate) 10 (41,6%), analgesic/antipyretic 8 (33,3%), anticonvulsant (carbamazepin) 3 (12,5%), and 3 (12,5%) patient unknown. There was a variety in the length of stay of hospitalization from 1 until 27 days with an average of 7,2 days.Systemic corticosteroid was the of choice against fatality, 19 (79,2%) recovered, 5 (20,8%) patient died, the cause of death were 3 (60%) broncopneumonia, 1 (20 %) septicemia and 1 (20 %) gastrointestinal bleeding.

Conclusion:The incidence of Steven Johnson Syndrome in Dr. Abdul Moeloek Hospital was found female is bigger than male, the highest group of age was 24-44 (54,1%) and the drugs most commonly involved were antibiotics (41,6 %) followed by analgesic/antipyretic (33,3%), broncopnemoni was the mostly cause of death (60%). 2007 World Allergy Organization

TOKSIK EPIDEMOLISIS NEKROTIKANDEFINISI : = penyakit yang berat yg ditandai dengan gejala kulit yg khas -> epidemolisis menyeluruh dapat disertai kelainan mukosa di orifisium dan mata

Etiologi :Alergi obat ~ SJS

Infeksi

Gejala Klinis :~ SJS yg beratProdromal : - sakit berat - demam tinggi - kesadaran menurun Kulit eritem generalisata -> vesikel, bula Mukosa erosi, eksoriasi krusta

Histopatologi : Epidermolisis

Nikolsky (+)

Pengobatan :~ Steven Johnson Syndrome

Prognosis : 50 - 70 % buruk Infeksi > baik dari obat Kematian 28,6 % Sindrom Stevens Johnson 1 %