Steven johnson syndrome & ten

STEVEN-JOHNSON SYNDROME & Toxic
Epidermal Necrolysis
Prof DR Dr Ariyanto Harsono SpA(K)

Difinition
Steven-Johnson Syndrome (SJS) is a collection of
clinical symptoms of mucocutaneous eruption
characterized by a triad of abnormalities in
vesicobulous skin, eyes and mucous orifice
accompanied by severe general symptoms.
Synonyms include: de Friessinger-Rendu
syndrome, erythema exudativum multiform
major, Poliform bullous erythema, Muko-
Cutaneo-ocular syndrome, dermatostomatitis
2Prof DR Dr Ariyanto Harsono SpA(K)

Pathophysiology
SJS etiology is difficult to determine with certainty, because
the cause of various factors, although in general is often
associated with immune response to drug. Some of the
factors causing SJS include: infection (viral, fungal,
bacterial, parasitic), drugs (Vaccine, salicylates, sulfa,
penicillin, ethambutol, Tegretol, tetracycline, digitalis,
contraceptives), food (chocolate), physical (cold air,
sunlight, X-ray), miscellaneous (collagen disease,
malignancy, pregnancy).

Pathophysiology…..
Pathogenesis of SJS is often associated with type III
hypersensitivity reactions (immune complex reaction)
induced by soluble complexes of antigen or its metabolites
with IgM and IgG antibodies and delayed-type
hypersensitivity reactions ( hypersensitivity reactions type
IV) is a reaction that is mediated by specific T
lymphocytes.
Prof DR Dr Ariyanto Harsono SpA(K) 4

Clinical Manifestation
Prodromal symptoms ranges from 1-14 days of
fever, malaise, cough, coryza, pain
swallowing, chest pain, vomiting, muscle
aches and arthralgia vary greatly in severity
and combinations of symptoms.

Clinical Manifestation…..
After that will arise in the lesions:
basic lesion is Erythema Exudativum multiforme, over 10% of
the body surface. There are several "target lesion" is a
pathognomonic symptom.
Form of skin erythema, papul, vesicles, or bullae
symmetrically on almost the entire body.
Mucosa in the form of vesicles, bullae, erosion, excoriation,
bleeding and red crustae. Bullae occur suddenly within 1-
14 days prodromal symptoms, appear on mucous
membranes, membranes of the nose, mouth, anorectal,
vulvovaginal area and urethral meatus. Ulcerative
stomatitis and crusting hemorrhagic is a prominent feature.

Target Lesion

Clinical Mabifestation…..
Eyes: catarrhalis conjunctivitis,
blepharoconjunctivitis, iritis, iridocyclitis, eyelid
edema and difficult to open, in severe cases
erosion and perforation of the cornea that can
lead to blindness. Ocular mucosa injury is the
trigger that causes ocular cicatricial pemphigoid,
a chronic inflammation of the ocular mucosa
which causes blindness. The time required from
onset until the occurrence of ocular cicatricial
pemphigoid vary from a few months to 31 years.

Clinical Manifestation…..
The basis of skin lesions is erythema exudativum multiforme
(EEM).
When extensive EEM <30% is called Erythema Exudativum
minor,
if> 30% Exudativum: Erythema multiforme major, when
accompanied by abnormalities in more than 2 mucosa:
Steven Johnson Syndrome (SJS). SJS be accompanied bullae,
 when <10% is called Steven Johnson Syndrome,
 when 10-30% bullae called Steven Johnson Syndrome-Toxic
Epidermal-Necrolisis (SSJ-TEN),
 when the bullae> 30% is called Toxic Epidermal Necrolisis
(TEN). 10Prof DR Dr Ariyanto Harsono SpA(K)

Basic lesion is erythema exudativum multiforme. Looks some
"target lesion".

Mucosal Lesion at mouth, eyes, genitals, and maybe anal
(minimal 3 mucosa)

Diagnosis
Diagnosis directed against manifestations according
to the triad of skin disorders, mucosa, eyes, and
its relationship with the pathognomonic target-
shaped lesions, or eye iris cattle, mucosal
abnormalities, fever. Also supported laboratory
tests such as examination of peripheral blood,
immunological examination, bacterial culture and
test the peripheral blood, and histopathological
examination of the skin biopsy.

Diagnosis…..
 Anemia can be found in severe cases with bleeding,
 leukocytes usually normal or slightly elevated,
 increase in eosinophils.
 Levels of IgG and IgM elevate,
 C3 and C4 normal or slightly decrease
 circulating immune complexes.
 A skin biopsy: there is no classic abnormality.
 Imunoflurosesensce can help diagnose of atypical
cases.

Differential Diagnosis
• The main differential diagnosis is
Staphylococcal scalded skin syndrome (4S)
and
• Toxic epidermal necrolysis (TEN) which is
almost similar clinical manifestations but the
general state of TEN looks worse than SJS. TEN
is the fact of heavy SJS.

Staphylococcal Scalded Skin Syndrome

Staphylococcal Scalded Skin Syndrome…..

Toxic Epidermal Necrolysis

TEN…..

Management
In general, patiants with SJS come up in general in
severe condition that therapy is usually given late:
Fluids and electrolytes, protein and calories
parenterally.
Broad-spectrum antibiotics, based on culture
results and subsequent resistance test preparation
germs of the skin lesions and blood.

Management…..
• Coticosteroid parenteral: dexamentasone initial
dose of 1mg/kg bolus, then for 3 days 0.2-0.5 mg /
kg every 6 hours. The use of systemic steroids is
still controversial, there is assume that the use of
systemic steroids in children can lead to slow
healing and significant side effects, but some are
considered favorable steroid and save lives.

Management…..
Antihistamines if necessary. Especially when
there is intense itching. Pheniraminehydrogen
maleate (Avil) can be given at a dose for ages 1-3
years 7.5 mg / dose, for ages 3-12 years 15 mg /
dose, administered 3 times / day. As for the dose
of cetirizine can be given to children aged 2-5
years: 2.5 mg / dose, 1 time / day;> 6 years: 5-10
mg / dose, 1 time / day.
Eyes as well as skin care and
Topical antibiotics

Management…..
Bullae in skin treated with wet compresses of
Burowi solution.
Not allowed to use topical steroids on skin
lesions.
Mouth Lesions were treated with Kenalog in
Orabase.
Secondary infection treated with antibiotic
therapy that rarely cause allergies, broad
spectrum, bactericidal and is not nephrotoxic,
such as intravenous clindamycin 8-16 mg / kg /
day intravenously, given 2 times / day.

Prognosis
In the cases that are not severe, the prognosis is
good, and healing occurs within 2-3 weeks.
Mortality ranged from 5-15% in severe cases
with complications or late and inadequate
treatment. Prognosis is more severe if there is
a wider purpura. Death is usually caused by
disorders of fluid and electrolyte imbalance,
bronchopneumonia and sepsis.

Toxic Epidermal Nekrolisis (TEN)
Toxic epidermal necrolysis, also known as TEN, is a
potentially deadly skin disease that usually resulting
from drug reactions. Such as Stevens-Johnson
Syndrome, the disease is usually a reaction to
medication. Both forms of the disease can be deadly
and extremely painful and distressing. Other drugs
associated with toxic epidermal necrolysis is
penicillin.
In many cases there is no known cause for TEN,
although the drug is the major cause.

 This condition can be very serious, and can cause discomfort
and pain, and in some cases the infection can be spread
through the disease result in death of the patient.
 Every age group can be affected by toxic epidermal
necrolysis. However, usually seen in older patients than
younger ones, and this is because older patients are more
likely to take medication, and therefore more likely to find
that they have a drug reaction. There are also other groups
that are more vulnerable when it comes to acquiring toxic
epidermal necrolysis. This includes those with AIDS, who have
low immunity to infection and disease.
 Parents and all other age groups are advised to be alert TEN
signs if they are in treatment.

Clinical Manifestation
Both toxic epidermal necrolysis and SJS can begin with non-
specific symptoms such as cough, pain, headache, and
fever. This may be followed by symptoms more specific
like a red rash on the face and trunk, which can continue
to spread to other parts of the body. The rash can form
into blisters, and blisters may form in areas such as the
eyes, mouth and vaginal area. Mucous membranes can be
inlflammed.TEN is heavy SJS. Bullae on TEN wider than
20%, a positive sign Nichosky may be noted.

Management
 Those suffering from TEN treated in the hospital, and if the
cause of the problem is drug related then the drug should
be discontinued. The patient was given intravenous fluids
to replace lost fluids, and the remaining skin to re-grow
itself. However, the chances of survival can vary depending
on the extent of damage and the degree of infection
experienced by the patient.
 500mg/kg dose IVIG with a single dose given per infusion
for 6-8 hours is very effective for TEN.

Those who were taking medications that can
cause TEN were advised to alert and able to
identify the warning signs associated with this
disease. Toxic epidermal necrolysis is a
potentially deadly.

Prof DR Dr Ariyanto Harsono SpA(K) 33Thank You

Steven johnson syndrome & ten

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