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