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SINDROM STEVEN JOHNSON

Disusun Oleh:
Muhammad Haidar Ilhamullah
Annelistiane
Hemriadi
Arini oktaviani

PEMBIMBING KLINIK:
dr. Nur Rahmah. S.M, M.Kes, Sp.KK
dr. Syahriani Syahrir, M.Kes, Sp.KK
Case
IDENTITY
Name : Mr. H
Age : 25 years
Sex : Male
Adress : Ds. Siboang dusun III
Marriage status : single
Date of examination: August, 11th
2016
ANAMNESIS
Main Complaints:
Scale skin whole of body.

History of present illness :


A man 25 years old was admitted to the hospital with
complaints of scale skin whole of body 1 months ago, after
he was consumed rifampicin, dapson and paracetamol. On
the first sign, his family just look at the patient have bites of
mosquito and then that bites has changed to be scale skin
whole of body. Patient with inflammatory of eyes, oral mucous
ulcertation, labial crusting, sore throat and feverish arthralgia.
In 1 month ago patient severe lose weight. May be find
many ulcer in other part of body. And painly if the patient
move his extremity. 1 week before arrive at hospital patient
with with any complaints fever and diarhea.
ANAMNESIS
History of previous disease :
Patient never have exprience like
this before
Diabetes and hypertensi (-)
Allergy history (-)
Treatment history : 2 months ago
he had a treatment with
Rifampicin and dapsone. 1 month
ago he had consumed
Paracetamol.
ANAMNESIS
Family history :
Member of family hasnt have
complain like patient
Physical examination
General status:
1. General situation: severe
2. Awareness : compous mentis
3. Nutritional status : Bad (LLA : 14 Cm)
4. Vital signs:
- Blood pressure : 110/60 mmHg
- Pulse : 90 x/minutes
- Temperature : 37,8C
- Breathing : 24x/minutes
Status dermatology
Localization : universal
Head : eyes : erythema
lips : diffuse erotion
Neck : thick skuama
Chest : thick skuama with erythema
erotion and ulcer
Stomach : thick skuama with
erythema erotion and ulcer
Status dermatology
Back : ulcer with thick skuama
Genitalia : thick scuama with
erythema and ulcer
Gluteal : ulcer with thick skuama
Upper extremitas : ulcer with
thick skuama
Lower extremitas : ulcer with thick
skuama
Resume
A man 25 years old was admitted to the hospital
with complaints of thick skuama whole of body 1
months ago, after he was consumed rifampicin,
dapson and paracetamol. On the first sign, his
family just look at the patient have bites of
mosquito and then that bites has changed to be
thick skuama whole of body.
Patient with inflammatory of eyes, oral mucous
ulcertation, labial crusting, sore throat and feverish
arthralgia. In 1 month ago patient severe lose
weight. May be find many ulcer in other part of
body. And painly if the patient move his
extremitas. 1 week before arrive at hospital fever
and diarhea.
Laboratory examination
Albumin : 1,6 g/dl
SGOT/SGPT : 19 UL/19UL
Urea : 76 mg/dl
WBC : 7,1/UL
RBC : 1,5/UL
HGB : 4.9 g/dl
Hematocrit : 96,7 %
Working Diagnosis

STEVEN JOHNSON SYNDROME (SJS)


Differential diagnosis
TEN (Toxic Epidermal Necrolysis)
Fixed drug eruption
Psoriasis vulgaris
Treatment
Non-medicamentose:
Compresed NaCL 0.9%
Repair general situation
Repair nutrition

Medicamentose

Systemic:
- Inj. dexamethasone /8H /IV
- Inj. Gentamicin /8H/IV
Topical :
-desoximethasone Cr 10 gr
-Gentamicin Cr 10 gr
Follow up Day care 1
Tgl Friday, August 12th 2016 (Day care 1)

S Thick Skuama with ulcer, erythematous, lip erotion difuse, conjungtivitis

O Vital Sign
BP. 100/60 mmHg Pulse 80x/minutes
Temperature 37,1 C Respiratory rate 20x/minutes
Head : eyes : erythema
lips : diffuse erotion
Universal : Thick Sskuama with erythema, erotion, and ulcer

Laboratory Examination
albumin (1.6 g/dl), SGOT/ SGPT (19 UL/19 UL), Urea (76 mg/dl) WBC
(7.1/UL), RBC (1.5/UL), HGB (4.9 g/dl), HCT (96.7 %)

A Sindrom steven johnson


P Non-Medikamentosa
Compresed NACL 0,9% (whole body)
Repair general ststus and nutritional
Medikamentosa
Sistemic
Inj. Dexamethasone. 1 Amp/8 H/ iv (D.1)
Inj. Gentamicin 1 Amp/8 H/ iv ( D.1)
Ringer Laktat 24 drips/ Mins
Topikal
Gentamicin cr 10 gr
Asam Salisilat 3%
Vaseline ad 30 gr

Anhydrous lanoline 60 mg
Isopropyl myristate 170 mg
Cetyl alcohol 20 mg
Sorbitan monostearate 80 mg
Polysorbate 80 mg
Glycerol 100 mg
Dimethicone 50 mg
Asam sorbic 1,5 mg
Day Care 1 Saturday, August
12th2016

Picture 1. Thick Skuama with ulcer, erythematous, lip erotion difuse, conjungtivitis
Follow up Day care 3
Tgl Saturday, August 13th 2016 (Day care 2)

S Thick Skuama with ulcer, erythematous, lip erotion difuse, conjungtivitis

O Vital Sign
BP. 100/60 mmHg Pulse 80x/minutes
Temperature 37,1 C Respiratory rate 20x/minutes
Head : eyes : erythema
lips : diffuse erotion
Universal : Thick Sskuama with erythema, erotion, and ulcer

Laboratory Examination
albumin (1.9 g/dl), SGOT/ SGPT (19 UL/19 UL), Urea (76 mg/dl) WBC
(11.1/UL), RBC (2.8/UL), HGB (8.4g/dl), HCT (86.3 %). HbsAG (Non-
reaktif), Anti HCV (Non reaktif), reaksi VDRL (-), Reaksi TPHA (Non
reaktif), Rapid I Test Anti HIV (Non reaktif).

A Sindrom steven johnson


P Non-Medikamentosa
Compresed NACL 0,9% (whole body)
Repair general ststus and nutritional
Medikamentosa
Sistemic
Inj. Dexamethasone. 1 Amp/8 H/ iv (D.1)
Inj. Gentamicin 1 Amp/8 H/ iv ( D.1)
Ringer Laktat 24 drips/ Mins
Topikal
Gentamicin cr 10 gr
Asam Salisilat 3%
Vaseline ad 30 gr

Anhydrous lanoline 60 mg
Isopropyl myristate 170 mg
Cetyl alcohol 20 mg
Sorbitan monostearate 80 mg
Polysorbate 80 mg
Glycerol 100 mg
Dimethicone 50 mg
Asam sorbic 1,5 mg
Day Care 3 Monday, August 14th
2016

Picture 2. Thick Skuama with ulcer, erythematous, lip erotion difuse, conjungtivitis
Follow up Day care 12
Tgl Saturday, August 23th 2016 (Day care 12)

S krusta, eritema on chest and abdominal, ulcer on regio occipital,,


Shoulder, back gluteal, upper and lower extremity
O Vital Sign
BP. 110/70 mmHg Pulse 80x/minutes
Temperature 36,6 C Respiratory rate 20x/minutes
Regio occipital : Ulcer
Generalisata : erythema with krusta, and ulcer

Laboratory Examination
albumin (2.32 g/dl), SGOT/ SGPT (19 UL/19 UL), Urea (76 mg/dl) WBC
(9.0/UL), RBC (2.8/UL), HGB (8.4g/dl), HCT (25.5 %)

A Sindrom steven johnson


P Non-Medikamentosa
Compresed NACL 0,9% (whole body)
Repair general ststus and nutritional
Medikamentosa
Sistemic
Inj. Dexamethasone. 1 Amp/8 H/ iv (D.1)
Inj. Gentamicin 1 Amp/8 H/ iv ( D.1)
Ringer Laktat 24 drips/ Mins
Topikal
Gentamicin cr 10 gr
Asam Salisilat 3%
Vaseline ad 30 gr

Anhydrous lanoline 60 mg
Isopropyl myristate 170 mg
Cetyl alcohol 20 mg
Sorbitan monostearate 80 mg
Polysorbate 80 mg
Glycerol 100 mg
Dimethicone 50 mg
Asam sorbic 1,5 mg
Day Care 12 Tuesday, August 23th
2016

Picture 3. ulcer on regio occipital, shoulder, back, and regio coxygeus


Day Care 12 Tuesday, August 23th
2016

Picture 4. krusta and erythematous on chest and abdominal


DISCUSSION
In this case the diagnosis of Steven
Johnson syndrome based on history and
clinical features. The history and clinical
symptoms of Steven Johnson syndrome
found in this case.
From Anamnesis and the
clinical finding
Complaints of scale whole of body 1 months ago,
after he was consumed rifampicin, dapson and
paracetamol. On the first sign, his family just look at the
patien have bites of mosquito and then that bites has
changed to be scale whole of body. Patient with
inflammatory of eyes, oral mucous ulcertation, labial
crusting, sore throat and feverish arthralgia. In 1 month
ago patient severe lose weight. May be find many ulcer in
other part of body. And painly if the patient move his
extremitas. 1 week before arrive at hospital fever and
diarhea.
DISCUSSION
Theory Case

StevensJohnson In this patient steven


syndrome (SJS) are johnson syndrome
acute life-threatening has found erythema,
mucocutaneous erotion, ulcer whole of
reactions characterized body.
by extensive necrosis
and detachment of the
epidermis. (Fitzpatrick's,
2008)
DISCUSSION
Theory Case
The overall incidence of Patients Mr. H is male
SJS and TEN was and is now 25 years
estimated at 1 to 6 cases old.
per million person-years
and 0.4 to 1.2 cases per
million person-years,
respectively.
(Fitzpatrick's. 2008)
Learning Case
Theory Case
EN is associated with high When the first day went
fever, pain, and weakness. into the hospital the
Visceral involvement is also patient was treated by
possible, particularly with thick skuama whole of
pulmonary and digestive
body.
complications.
(Fitzpatrick's,2008)
Learning Case
Theory Case
Mucous membrane When the second day of
involvement (nearly always treatment in hospital
on at least two sites) is care in the first days of
observed in approximately erythema with thick
90% of cases and can
skuama whole of body.
precede or follow the skin
eruption. It begins with
erythema followed by painful
erosions of the oral, ocular,
and genital mucosa.
( Fitzpatrick's. 2008)
Discussion
From the results of the history and
physical examination and an explanation
theory in case then this dignosis patients
with steven johnson syndrome. Steven
johnson syndrome is caused by the The
pathophysiology of epydermal necrolysis
is still unclear; however, drugs are the
most important etiologic factors.
(Fitzpatrick's, 2008).
Discussion

However, it is generally accepted that


specific and nonspecific cytotoxic cells
are too few within the lesions to explain
the death of cells on the full thickness
and large areas of the epidermis and
mucous membranes. (Fitzpatrick's,
2008).
Discussion

Figure 2. The human body sensory dermatomes


Discussion

Figure 2. Clinical
finding
In this patient steven johnson syndrom is
Discussion
dormant Only in very rare cases with prior
reaction and
inadvertent rechallenge with the same drug
does it
appear more rapidly, within a few hours.
Nonspecific
symptoms such as fever, headache, rhinitis,
cough,
or malaise may precede the mucocutaneous
lesions
by 1 month .(Fitzpatrick's, 2008).
Differential Diagnosis
-Toxic Epidermal Necrolysis
-Fixed Drug Eruption
-Psoriasis Vulgaris
Therapy in patients
CORTICOSTEROIDS. The use of systemic
corticosteroids is still controversial. Some
studies found that such therapy could prevent
the extension of the disease when
administered during the early phase,
especially as intravenous pulses for a few day.
INTRAVENOUS IMMUNOGLOBULIN. The
proposal to use high-dose intravenous Ig was
based on the hypothesis that Fas-mediated cell
death can be abrogated by the anti-Fas activity
present in commercial batches of normal
human Ig.
CYCLOSPORINE A. Cyclosporine is a powerful
immunosuppressive agent associated with
biologic effects that may theoretically be
useful in treatment of EN: activation of T
helper 2 cytokines, inhibition of CD8+
cytotoxic mechanisms, and antiapoptotic
effect through inhibition of Fas-L, nuclear
factor-B, and TNF-. Several case reports
and series suggested some efficacy of
cyclosporine A in halting the progression of
EN without
worrisome side effects when administered
early
MUCHO
GRACIAS

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