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Case Report

SINDROM STEVENS JHONSON

SUPERVISOR :
Dr. Nur Rahmah M.kes,Sp.KK
Dr. Sukma Anjayani M.kes,Sp.KK
Dr. Syahriani M.kes, Sp.KK

ANAMNESIS
Main complaints
Fever

History of present illness :


Male patients 34 years old Anutapura hospital with
complaints raddish rash on the entire body
experienced since 8 days ago. Intially the patients has
a fever and got Paracetamol and vitamin from the
clinic. After that the patients has an appear reddish
rash and itchy. Complaints aggraveted after peeling
lips like a blister. Red eyes and difficult open the eyes.

ANAMNESIS
History of previous disease:
Patients had never experienced the
same thing with the scenario.
No member of the family who have
the same complaint with the patient

Physical Examination
General Status:
1. General Situation : mild
2. Awareness : compous mentis
3. Nutritional status : Good
4. Vital Signs :
a. Blood pressure : 100/80 mmHg
b. Pulse : 76x / min
c. Temperature : 37oc
d. Breathing : 20x / min

Status Dermatology
Localization
Regio
Size
Lesion
a. Lips
b. Body

: Spread on the whole body


: Facialis, thoracalis, upper and lower limb, back, upper and lower leg.
: Miliar up to lentikuler
:
: Erytheme vesicles, erotion, and crust.
: Multiple papul with erythematous base.

Status Dermatology
Size
: lenticular and
numular
Lession :
In the lips erytheme vesicles,
erotion, and crust, body
multiple
papul
with
erythematous base.

Differential Diagnosis
1. TEN (Toxic Epidermal Necrolisys)

Working
Diagnosis

SINDROM STEVENS
JHONSON

Treatment
1. Topical :
- Kenalog in orabase
2. Sistmik :
- NaCl 9%
- Deksametason intravena 4-6 x 5 mg sehari
- Prednison 30- 40 mg sehari
- Cyclosporin 5 mg/KgBB/day
- Immunoglobulin 3 gr/KgBB/day

Follow up Day care 1


Tgl

Tuesday, May 24th 2016 (Day care 1)

Reddsh rash on the entire body, crusta in the lips, conjungtivitis

Vital Sign

A
P

Sindrom steven johnson


Topical
- Kenalog Cream in lesion lips
- Inerson 15 gr + Fuson cream in body (morning afternoon)
- compress NACL 0,9

BP. 100/70 mmHg


Pulse 80x/minutes
Temperature 36 C
Respiratory rate 20x/minutes
Localization Facialis, thoracalis, upper and lower limb, back, upper and
lower leg.
Lession of orbita : erytheme,erotion difus
Lesion of lips
: Vesicle erytheme, erotion difus, crust.
Lesion of body
: Multiple papul with erythematous base, round shape,
sirkumskrip, miliar up to lentikuler.

Systemik
- Dexamethasone Injection i/12 hours day IV
- Gentamicyn Injection/12 hours day IV
- Ringer Laktat 24 drops/ Minute

Day Care 1

Vesicle erytheme,
erotion difus,
crusta.

Tuesday, May 24th 2016

Multiple papul with


erythematous base, round
shape, sirkumskrip, miliar up
to lentikuler.

Follow up Day care 2

Tgl

Wednesday, May 25th 2016 (Day care 2)

Reddsh rash on the entire body, crusta in the lips, conjungtivitis

Vital Sign
BP. 110/70 mmHg
Pulse 80x/minutes
Temperature 36 C
Respiratory rate 20x/minutes
Localization Facialis, thoracalis, upper and lower limb, back, upper and
lower leg.
Lesion of lips
: Vesicle erytheme, erotion difus, crusta.
Lesion of body
: Multiple papul with erythematous base, round shape,
sirkumskrip, miliar up to lentikuler.
Sindrom steven johnson

A
P

Topical
- Kenalog Cream in lesion lips
- Inerson 15 gr + Fuson cream in body (morning afternoon)
- compress NACL 0,9
Systemik
- -

Dexamethasone Injection i/12 hours day V

- Gentamicyn Injection/12 hours day V


- Ringer Laktat 24 drops/ Minute

Day Care 2

Vesicle erytheme,
erotion difus,
crust.

Wednesday, May 25th


2016

Multiple papul with


erythematous base, round
shape, sirkumskrip, miliar up
to lentikuler.

Follow up Day care 3

Tgl

Thursday, May 26th 2016 (Day care 3)

Reddsh rash on the entire body, crusta in the lips, conjungtivitis

Vital Sign
BP. 110/70 mmHg
Pulse 80x/minutes
Temperature 36 C
Respiratory rate 20x/minutes
Localization Facialis, thoracalis, upper and lower limb, back, upper and
lower leg.
Lesion of lips
: Vesicle erytheme, erotion difus, crust.
Lesion of body
: Multiple papul with erythematous base, round shape,
sirkumskrip, miliar up to lentikuler.
Sindrom steven johnson

A
P

Topical
- Kenalog Cream in lesion lips
- Inerson 15 gr + Fuson cream in body (morning afternoon)
- compress NACL 0,9
Systemik
- Dexamethasone Injection i/12 hours day I
- Gentamicyn Injection/12 hours day VI
- Ringer Laktat 24 drops/ Minute

Day Care 3

Thursday, May 26th


2016

Vesicle erytheme,
erotion difus,
crust.

Multiple papul with


erythematous base, round
shape, sirkumskrip, miliar up
to lentikuler.

Follow up Day care 4

Tgl

Friday, May 27th 2016 (Day care 4)

Reddsh rash on the entire body, crusta in the lips, eyes can open

Vital Sign
BP. 110/70 mmHg
Pulse 84x/minutes
Temperature 36 C
Respiratory rate 20x/minutes
Localization Facialis, thoracalis, upper and lower limb, back, upper and
lower leg.
Lesion of lips
: Crust.
Lesion of body
: Multiple papul with erythematous base, round shape,
sirkumskrip, miliar up to lentikuler.
Sindrom steven johnson

A
P

Topical
- Kenalog Cream in lesion lips
- Inerson 15 gr + Fuson cream in body (morning afternoon)
- compress NACL 0,9
Systemik
- Dexamethasone Injection i/12 hours day II
- Gentamicyn Injection/12 hours day VII
- Ringer Laktat 24 drops/ Minute

Day Care 4

Friday, May 27th 2016

crust in the lips, eyes can open

Follow up Day care 5

Tgl

Saturday, May 28th 2016 (Day care 5)

Reddsh rash on the entire body, crusta on the lips began to decrease

Vital Sign
BP. 100/70 mmHg
Pulse 80x/minutes
Temperature 36 C
Respiratory rate 20x/minutes
Localization Facialis, thoracalis, upper and lower limb, back, upper and
lower leg.
Lesion of lips
: Crust.
Lesion of body
: Multiple papul with erythematous base, round shape,
sirkumskrip, miliar up to lentikuler.
Sindrom steven johnson

A
P

Topical
- Kenalog Cream in lesion lips
- Inerson 15 gr + Fuson cream in body (morning afternoon)
- compress NACL 0,9
Systemik
-

Methylprednisolon 4-3-0 Day I

- Gentamicyn Injection/12 hours day VIII


- Ringer Laktat 24 drops/ Minute

Day Care 5

Saturday, May 28th


2016

crust on the lips began to decrease

RESUME
Mr. U patients 39 years of age entered Anutapura hospital with with
complaints raddish rash on the entire body experienced since 8 days
ago. Intially the patients has a fever and got Paracetamol and vitamin
from the clinic. After that the patients has an appear reddish rash and
itchy. Complaints aggraveted after peeling lips like a blister. Red eyes
and difficult open the eyes. There is no previous history of the disease
and no family history of disease.
On physical examination, the patient's status generalist awareness
compos mentis, BP (100/80 mmHg), temperature (37 C), respiratory
(20x / min), pulse (76x/min). For the status of dermatology obtained
intentions skin disorders in the eye there is erythema and erosion
diffuse, on the lips obtained vesicular erythema and erosion diffuse
accompanied by crusta, and on the neck, chest, abdomen, back,
upper extremities, lower extremities obtained papules multiple, round
shape , size milliar and lenticular, with circumscribed limits.

DISCUSSION
In this case the diagnosis of
Sindrom stevens Jhonson based on
history and clinical features. The
history and clinical symptoms of
Sindrom Steven Jhonson found in
this case

From Anamnesis and the


clinical finding
Complaints of fever accompanied by red
spots all over the body since 8 days ago.
Previously, patients had a fever and had
been taking medication for fever
(Paracetamol) are obtained after treatment
to a clinic. After that, came the red patches
all over the body accompanied by intense
itching and heat. Complaints aggravated
after the patient's lips and peeling like a
blister. Red-eye patients and patients with

DISCUSSION
Theory

Case

Stevens-Johnson
syndrome
is
an
immunecomplexmediated hypersensitivity
complex that typically
involves the skin and the
mucous membranes.

In
these
patients
Stevens

Johnson
syndrom occurs in the
skin and mucosa.

DISCUSSION
Theory

Case

2 cases per million Patients Mr.U is male


people per year
and is now 34 years
Females comprise 33- old
62% of cases
Age : 25 47 years

Learning Case
Theory

Case

The above in accordance with the theory that


although patogenesis EN connected with drugs
eruptions. At the inception of the lessions occur
dase reaction cell-mediated cytotoxic (cellmediated cytotoxic) against keratinocytes cause
massive apoptosis. Immunopatologis research
showing their CD8+ T Lymphocytes in the
epidermis and dermis skin exposed tang, has an
activity of lymphocytes sch as natural killer cells
in early lessions. The CD8+ T Lymphocytes
expressing TNF and TNF receptor on the
surface of cells that are capable of destroying
cells through perforin. And the clinical cutaneus
lesion is erhytheme,dusky red and purpuric
macules. Extracutaneus symtoms is fever, pain
and weakness. Mucous membrane involvement
is eritema followed by pain erosions of the oral,
ocular and genital mucosa.

When the patient and family


history and originally had a
fever-lowering medication hot
but a few hours later the
symptoms arise like the above.
Patient and family expressed
no history of any drug
allergies, and often take
medication paracetamol and of
drugs allergies patients also
ranitidine and ketorolac.

The immunologic pattern of early lesions


Cell-mediated cytotoxic reaction
Keratinocytes leading to massive apoptosis
Immunopatologis research showing their CD8+
T Lymphocytes in the epidermis and dermis skin
Exposed tang
Amplification by cytokind
TNF- & soluble Fas ligand (Fas-L)
Only granulysin & to much lesser degree perforin,
were able to destroying cell keratinosit in vitro
Clinical and histological lesion

PATOMEKANISM OF
EPIDERMAL
NECROLISIS

Learning Case
Theory

Case

The
eruption
is
initially
symmetrically distributed on
the face, the upper trunk, and
the proximal part of limbs. The
initial
skin
lesions
are
characterized by erythematous,
dusky red, purpuric macules,
irregularly
shaped,
which
progressively
coalesce.
It
begins with erythema followed
by painful erosions of the oral,
ocular, and genital mucosa.

When
first
admitted
to
hospital
pattients
were
admitted with symptoms of
fever, spot appear all over
the body, puffy lips and
vesicle and pain the eyes.
These symptoms appear after
consuming
the
drug
Paracetamol.

Diagnosis :
1. Anamnesis
. History taking medication before symptoms
appear
. Clinical Symptomps
2. Physical Examination
. Cutaneus Lession: Erythematous , Vesicles , Bulla
. Mucous membrane involvement : It begins with
erythema followed by painful erosions of the oral,
ocular, and genital mucosa.
. Conjunctivitis

Histopatologi

Discussion
From the results of the history and physical
examination and an explanation theory in
case then this dignosis patients with Stevens
Johnson syndrom.

Discussion

Discussion
Cliniycally begins within 8 weeks (usually 4 to
30 days) after the onset of drug exposure for
the firs time. The eruption is initially
symmetrically distributed on the face, the
upper trunk, and the proximal part of limbs.
The initial skin lesions are characterized by
erythematous, dusky red, purpuric macules,
irregularly shaped, which progressively
coalesce. It begins with erythema followed by
painful erosions of the oral, ocular, and

Discussion

Figure 1. Extensive erosions and necroses of the lower lip and oral
mucosa, massive erosions covered by crusts on the lips.

Discussion

Extensive erosions and necroses of


the lower lip and oral mucosa.

Differential Diagnosis
TEN (Toxic Epidermal Necrolisys)

Therapy in patients
Topical Treatment
steven johnson syndrome treatment based on
the patient's general condition
Topical treatment:
Kenalog in orabase At lip sores
Systemic treatment
Cyclosporin A 5mg/KgBB/day
Intravenous Immunoglobulin 3gr/KgBB/day


Antibiotics have rarely cause
allergies, bactericidal properties and
no or little nephrotoxic (ciprofloxacin
2x400 mg intravenously / 24 hours).

Dextrose 5%, NaCl 9% 1:1


Complication
The most common complications
of blindness due Iyalah lacrimation
disorder.

Prognosis

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