M.

Syafei Hamzah
SMF/Bagian Kulit dan Kelamin
RSUD Dr.H. Abdul Moeloek,FK Unila Lampung
1. NEKROLISIS EPIDERMAL :
- SINDROMA STEVENS-JOHNSON (SSJ)
- NEKROLISIS EPIDERMAL TOKSIKA (NET)
2. PEMFIGUS VULGARIS
3. ERITRODERMA (Dermatitis ekfoliativa




- PENDERITAAN
- KECACATAN AKIBAT KOMPLIKASI
- KEMATIAN



-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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ARTICLE LINKS:
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World 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. 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 berat
Vesikel atau bula yang kendur , mudah
pecah, erosi, hiperpigmentasi dan krusta
Mukosa 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
Nikolskys sign
Asboe-Hansens sign (spread phenomen)

Histopathology :
- acantholytic cells (Tzancks 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