M.Syafei HamzahSMF Kulit dan Kelamin
RSUD Dr.H. Abdul Moeloek Lampung
1.NEKROLISIS EPIDERMAL
-SINDROMA STEVENS-JOHNSON (SSJ)
- NEKROLISIS EPIDERMAL TOKSIKA (NET)
SJS TEN
Epidermal Necrolysis
SIMILAR in:• clinical findings• histopathology• drug etiology• mechanisms
DIFFER in:% BSA
involved
SJS TEN
DEFINISI : = kumpulan gejala penyakit pada kulit dan mukosa disertai gejala sistemik, -> Eritema multiforme berat ; bersifat akut, ditandai trias -> kulit, mukosa, mata.
- insiden 1,2 – 6 per sejuta tiap th - tidak ada perbedaan ras - semua umur
-> pasti belum diketahui.1. Alergi obat >> a.l. sulfa, penisilin, NSAID, antikonvulsi, antipiretik, jamu.
2. Infeksi -> virus, bakteri,parasit neoplasma, vaksinasi, radioterapi
Pasti -> tidak diketahui reaksi hipersensitivitas tipe III & 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
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
-> mulut, faring, alat genital - vesikel, bula -> erosi,
eksoriasi -> krusta kehitaman
Kelainan mata : -> konjuctivitis kataralis
>> konjungtivitis purulen,
perdarahan,simblefaron ulkus kornea, iritis
Tergantung berat – ringan . Rawat, Keadaan umum, cepat -> life saving-> kortikosteroid - Methyl Prednisolone 125 – 250 mg/hari (deksametasone : 4-6 x 5mg/hari) -> tappering off segera. - Antibiotika - intake cairan/elektrolit - Reseptor H 2 Ranitidin
Bronkopneumoni Syok Kelainan ginjal -> ATN Kebutaan
Bertindak cepat dan tepat ->prognosis baik
Kematian -> 5 - 15 %
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PDF (150 K) | View full-size inline images World Allergy Organization Journal:Volume WAC 2007 AbstractsNovember 2007p S276
Stevens Johnson Syndrome in Bandar Lampung, Indonesia [Abstracts: Abstracts of the XX World Allergy Congressâ„¢ 2007 December 2-6, 2007, Bangkok, Thailand: POSTER GROUP 3 -
WEDNESDAY: DRUG ALLERGY: 863] Hamzah, M. Syafei Dr. 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
DEFINISI : = penyakit yang berat yg ditandai dengan gejala kulit yg khas -> epidemolisis menyeluruh dapat disertai kelainan mukosa di orifisium dan mata
Alergi obat ~ SJS
Infeksi
~ SJS yg berat Prodromal : - sakit berat - demam tinggi - kesadaran menurun Kulit eritem generalisata -> vesikel, bula Mukosa erosi, eksoriasi – krusta
Epidermolisis
Nikolsky (+)
~ Steven Johnson Syndrome
Prognosis : 50 - 70 % buruk
Infeksi > baik dari obat Kematian 28,6 % Sindrom Stevens Johnson 1 %
Definisi : = suatu penyakit kulit dan mukosa yang ditandai dengan bula intradermal yang kronik .
Kulit bisa normal atau eritem
• Laki-laki = perempuan
• Umur : 50 – 60 tahun
• Anak-anak jarang
• Etiologi : ?
• Drugs : penicillamine, sulfhidryl,
captopril,penicillin,rifampicin,etc.
Keadaan umum : jelek spt sakit beratVesikel atau bula yang kendur , mudah pecah, erosi, hiperpigmentasi dan krustaMukosa mulut dan esofsagus juga terkena
sakit menelan
Predilesi : mukosa mulut, kepala punggung, bokong. Muka, leher, lengan dan geniatalia
auto-immune mechanism, acantholysis, intra epidermal
Antibody binding surface cell glycoprotein
plasmin and enzyme activity
Intercellular cement damage
Nikolsky’s signAsboe-Hansen’s sign (“spread phenomen”)
Histopathology : - acantholytic cells (Tzanck’s method) - intra epidermal blister - IgG dermoepidermal junction (immunofluorescence)
• Berat diopname
• Kortikosteroid ~ gejala klinik - methyl prednisolon : 125 mg – 250 mg tappering off - antibiotika - immunosupressive - cyclophosphamide
• Quo ad vitam, high mortality rate
• Quo ad functionam, dubia
• Quo ad sanationam, dubia
= Penyakit kulit yang ditandai dg
eritema difus dan skuama > 90%
permukaan tubuh disertai gejala
sistemik
1. Perluasan dari penyakit kulit seperti : PSORIASIS, DERMATITIS ATOPIK, DERMATITIS
SEBOROIK
2. Alergi obat
3. Perluasan dari penyakit sistemik
Keganasan (Sezary syndrome)
Makula eritem dan skuama generalisata
LABORATORiUM &
HISTOPATOLOGI
~ ETIOLOGY
Penderita rawat inap
Sistemik : Kortikosteroid
Topikal : EMOLLIENT
PROGNOSIS ~ ETIOLOGY