KULIAH SMT V

BY : KETUT SUTEJA WIBAWA

1. STEVENS JOHNSON SYNDROME
2. TOXIC EPIDERMAL NECROLYSIS
3. ERYTHRODERMA
4. ERYTHEMA MULTIFORME
5. STAPHYLOCOCCAL SCALED SKIN
SYNDROME (SSSS)
STEVENS-JOHNSON
SYNDROME
Stevens-Johnson Syndrome

(SJS)  episodic acute

mucocutaneous intolerance

reactions most often elicited by

drugs & less so by infections.

 Insidens

• 1- 2 per million / year.

• Female twice > males.

• Adult > children.

 Etiology

 > 50%  drug is causative

factor.

 Minority  infections,

vaccination etc.
 Pathogenesis

Hypersensitivity reaction

III & IV type.

 Clinical Feature

Trias sign :

1. Skin.

2. Mucous-membrane.

3. Eye.
It begins nonspecific prodrome :
• Fever • Malaise
• Headache • Rhinitis
• Cough • Sore throat
• Chest pain • Vomiting
• Diarrhea • Myalgia
• Arthralgia.
Skin :

• Erythematous (sometimes

morbiliform rash).

• Vesicle.

• Bullous, pustular  rarely.

Mucous membrane :
Two mucous surface minimize
• Lips.
• Oral cavity (palate,
buccal).
• Anogenital.
Sign :
• Erythema.
• Edema followed blister that
rupture & transform into
extensive.
• Hemorrhagic dull red erosions
coated by grayish-white
pseudomembrane or shallow
aphthous-like ulcers.
Oral lesions  painful, cause
eating difficult & hyper-
salivation.

Genital  painful hemorrhagic

bullous-erosive or purulent
lesions.

Anal  erosi.
Eye : Conjunctiva 

• Inflammation & chemosis.

• Vesiculation & painful

erosions.

• Bilateral lacrimation.
• Purulent conjunctivitis with

photophobia &

pseudomembran.

• Corneal ulceration, anterior

uveitis & panophthalmitis.

 Histopathology

• Satellite-cell necrosis (early

stages)  epidermal 

eosinophilic necrosis of the

basal & suprabasal layers 

subepidermal separation.
• Mononuclear cell infiltrate

 papillary dermis.

• Exocytosis  epidermis.
 Laboratory

• Blood sedimentation rate .

• Leucocytosis.
• Fluid-electrolyte imbalance.
• Microalbuminuria, hypo-
proteinemia
• Liver transaminase ,
anemia.
 Diagnosis

Trias sign :

• Skin, mucous-membrane, eye.

• < 10% or 10 – 30% body

surface area involvement.

 Differential Diagnosis

• Erythema multiforme : < 10%

lesion of the body surface +
typical target lesion localized.
• SSSS : caused by
staphylococcal epidermolisyn
toxynemia subcorneal
acantholysis.
• Macular drug eruption.

• Fixed drug eruption.

• Acute GVHD.

• Viral exanthems.
 Complication

Toxicity, dehydration, water &

electrolyte imbalance 
hemodynamic shock.

Pulmonary edema, mental

obtusion, confusion, coma &
seizure.
 Late Complication
• Skin of heal : hyper and/ or
hypopigmentation.
• Mucosa : scarring.
• Eye :
 Symblepharon, synechiae
 corneal opacities or
scarring  blindness.
 Treatment
• According cause, type, stage &
complications.
• Corticosteroid :
 Not be used routinely.
 Early stage of drug induced
SJS.
 Prednisone 1 – 2 mg/kgBB/d
or dexamethasone 4 x 10
mg/d/i.v.
• Antibiotic :
 Prophylactic  prevention
infection.
 According result culture 
skin, mucous erosion.
e.g. gentamycine 2 x 60 mg <
40 kg body weight, 2 x 80 mg
> 40 kg body weight 
monitoring renal function /
week.
• Monitoring Hemodinamik,

blood gases & fluid,

electrolytes & protein

balance.
• Supportive care :

 Pulmonary care (suctioning,

postural drainage, etc).
 Ophthalmologic care.
 High-calorie & high protein
diet.
• Topical treatment :

 Sofratulle / sulfadiazine

cream.

 Kenalog in orabase  oral

lesion.
 Prognosis

Mortality rate  severity

disease & medical care.

TOXIC EPIDERMAL
NECROLYSIS
(TEN)
 DEFINITION

The disease that characterized by:

 Rapidly expanding macular
rashes of more than one
mucosal site.
 The rash coalesces to
widespread erythema, necrosis,
& bullous detachment of the
epidermis resembling scalding.
INCIDENCE & EPIDEMIOLOGY

1. The average incidence at 0.5 to

1.4 per million per year.
2. There is no ethnic
preponderance; females appear
to be about twice as frequently
affected as males.
3. It is most often found in
adults.

4. It typically occurs
sporadically, but epidemics
have been observed with
the mass use of drugs.
 ETIOLOGY
It is a polyetiologic reason pattern :

 Drugs (the leading causative

factors).
 Infection.
 Vaccination.
 Graft versus host disease
(GVHD).
 PATHOGENESIS

Cytotoxic immune reaction Cell T (CD4

& % CD8)
TNF α

Inducing apoptosis

destruction of epidermis &
keratinocyte
CLINICAL FEATURES

 TEN begins with a

nonspecific prodrome of 1

to 14 days in at least half of

patients.
 A macular at times morbiliform
rash appears first on the face,
neck, chin, & central trunk
areas & may then spread to the
extremities & the rest of the
body.
 The lesions rapidly increase in
numbers & size : maximal
disease expression is usually
reached within 4 to 5 days.
 The rash is paralleled or even
preceded by mucous membrane
lesions.
 Extra cutaneous symptoms :
 Toxicity, dehydration, &
water & electrolyte
imbalance may proceed to
hemodynamic shock,
pulmonary edema, coma,
etc.
 Late complications :
 Skin lesions heal with transitory
hyper- and / or hypopigmentation
 Scarring of mucosal lesions, which is
most serious in the eyes.
 A Sjogren like Syndrome
(anautoimmune disease characterized
by dryness of the mucous membrane
of eyes, nose, mouth & vagina).
 In small minority of case,

TEN presents with primary ill-

demarcated diffuse
erythemas that are rapidly
progressive & may become
erythrodermic.
 HISTOPATHOLOGY

Erythema multiforme with

extensive eosinophilic necrosis

of the epidermis & cleavage

plane above the basement

membrane.
 LABORATORY
INVESTIGATIONS

 An elevated blood

sedimentation rate.

 Moderate leukocytosis,

anemia.
 Fluid-electrolyte imbalances,
microalbuminuria,
hypoproteinemia.
A transient decrease of
peripheral CD4+ T lymphocyte
counts.
 DIAGNOSIS

Tzank preparations showing

cuboidal cells & skin biopsy can

be used to confirm the

diagnosis.
DIFFERENTIAL DIAGNOSIS

 Staphylococcal scalded skin

syndrome.
 Generalized fixed drug
eruption.
 Burns, cauterizations, etc.
 Toxic erythroderma.
 TREATMENT

 Systemic glucocorticoids 

80 to 120 mg of methyl-

prednisolone per day until

disease progression has

decreased.
 Prophylactic antibiotic

treatment should be started

right from the beginning.

Sulfonamides & antibiotics with

known sensitizing potential must

be avoided (aminopenicilline,

cephalosporins).
 Topical treatment may be
carried out with hydrocolloid or
more conservatively, with
gauze dressing.
Obviously, sulfonamide-
containing topical agents
should be avoided.
 PROGNOSIS

 Severe morbidity & high

mortality.
 Death is usually due to :
 Sepsis.
 Gastrointestinal hemorrhage.
 Renal, hepatic or pulmonary
complications.
ERYTHRODERMA
 Definition

 Inflammatory skin disease

characterized by erythema
which affects more than
90% of the body surface.
 Usually followed by
exfoliation of scale.
 Synonym

Exfoliative dermatitis
 Etiology
 Drug reaction.
 Expansion of other skin
disease i.e psoriasis,
seborrhoeic dermatitis,
atopic dermatitis.
 Systemic disease or
malignancy.
 Unknown.
 Pathophysiology

 Erythema  disorder of

thermoregulation  basal

hypermetabolism.

 Scaling  lost of protein

 hypoalbuminemia.
 Clinical Manifestation

I. Erythroderma due to drug

reaction.
Acute, occurs within 10 days
after the suspected drug
administrated.
The eruption start as
generalized morbiliform
erythema, initially without
scale.
II. Erythroderma as an
expansion of other skin
disease.

Characterized by exfoliative
& desquamative skin
lesions.
 Psoriatic erythroderma

(most common in adults).

 Leiner’s disease (in infant

4 - 20 weeks of age).
III. Erythroderma due to systemic
disease or malignancy.
 Hodgkin’s disease &
mycosis fungoides / sezary
syndrome.
 Universal erythema,
accompanied by scale &
severe pruritus.
 Laboratory

 Type I & II : not specifically,

but occasionally found
leucocytosis & increased
serum IgE.
 Type III (sezary syndrome) :
sezary cells > 1000/mm3.
 Histopathology

The histological appearances

vary depending upon the

severity an duration of

inflammatory process :
I. Acute stage (type I) :
 Spongiosis.
 Parakeratosis.
 Non specific inflammatory
infiltrate.
II. Chronic Stage :
 Acanthosis.
 Elongation of rete ridges.
III. Type III :
 Lymphoid infiltrate at
dermo-epidermal junction.
 Atypical cerebriform
mononuclear cells.
 Pautrier’s micro-abscesses.
 Diagnosis

 Anamnesis.

 Clinical features.

 Histopathology.
Differential Diagnosis

 Pemphigus foliaccus.

 Pityriasis Rubra Pilaris.

 Treatment

Depend on the causative factor.

1. Supportive treatment (for all

types) :
 Hospitalization.
 Fluid & elctrolyte balance.
 High protein intake.
2. Systemic treatment :

prednisone dose.

 3 - 4 x 10 mg/d  type I.

 4 x 10 - 15 mg/d  type II

(adult).
 30 mg/d + Chlorambucyl 2

- 6 mg/d  type III.

 1 - 2 mg/kgBW  Leiner’s

disease.
3. Topical treatment :

 Triamcinolone acetonide

cream (Type I, II).

 Psoralen + UVA (Type II).

 Potent topical steroid

cream (type II).

 Nitrogen mustard cream

(Type III) .
Depend on the causative factor
 Type I : Although can be fatal
at the acute phase, it has the
best prognosis if treated with
adequate treatment. Often
resolving in 2 – 6 weeks.
 Type II : relapses often
occur.
 Type III :

 Poor prognosis.

 Less than 1 year Median

survival rates.
 ERYTHEMA
MULTIFORME
Erythema multiforme (EM) is a

disease spectrum that

comprises a group of acute

self limited exanthematic

intolerance reaction.
• Von Hebra descriptions EM
associated HSV.
• Steven & Johnson as EM
linked SJS because the same
pathologic, differ only in
severity & term EM minor &
major.
• EM major synonym SJS.
 Two main subset

1. EM - a fairly common, usually

mild & relapsing eruption that

is most often triggered by

recurrent HSV infection.

2. SJS - TEN complex an

infrequent severe muco-

cutaneus intolerance

reaction most often elicited

by drugs.
Incidence & Epidemiology
• Relatively common.
• Can be observer in all ages,
predominantly in adolescent
& young adult.
Rare in under 3 years olds &
over 50 years.
• Female = male.

No predominance for ethnic

groups.
• Often recurrent in short
interval & reappear for many
years.
 Etiology

• Triggered by HSV-1 & HSV-2.

• Drugs as rare cause of EM.

 Pathogenesis

• A cell mediated immune

reaction aimed at the

destruction of keratocytes

expressing HSV antigens.

• EM associated SJS-TEN is
characterized by a dense
dermal inflammatory infiltrate
that is composed chiefly of
CD4+ T lymphocyte &
monocyte  the wheal-like
clinical appearance of the
typical target lesion.
Clinical Manifestation

• Lesion appear within 3

days.

• Up to hundred of lesion

may form.
• Symmetric, extensor surfaces

of the extremities & face

(centripetal).

• Less often on palms & soles,

thigh, bottocks & trunk.

• Usually symptomless,
sometimes burning & itching.
• Typically, the lesion is a highly
regular circular, wheal-like
erythematous papule or plaque
that is stable with classic target
as iris lesion.
• Target lesion consist : a
dusky central disk (blister),
more peripherally a ring of
place edema & erythematous
halo.
• Not all lesion are typical.
 Pathology

• Early :

 Lymphocyte accumulation at

the dermal - epidermal

interface with exocytosis into

the epidermis.
 Scattered keratinocyte necrosis

with lymph attached to the

necrotic keratinocye (satellite-

cell necrosis).
 Spongiosis, vacuolar

degeneration of the basal

layer.

 Focal junctional & sub-

epidermal cleft formation.

• Advanced : subepidermal

blister formation &

epidermal necrosis.
Differential Diagnosis
• Acute annular urticaria.
• Urticaria vasculitis.
• Disseminated lesion of
contact dermatitis.
• Bullous pemphigoid.
• Linear IgA dermatosis.
• Herpes gestationes.
 Treatment
• Symptomatic : shake lotion,
topical steroid, analgetic &
anti histamin.
• Systemic glucocorticoids :
Unnecessary & possibly
worsened.
• Because recurrent EM most
often by triggered HSV
infection  the ideal
approach : prevention of HS
episodes with oral acyclovir
or derivates.
Alternatives Treatment

• Dapsone.

• Anti malaria.

• Azathioprine.

• Thalidomide.
 Prognosis
• Self limited, recovery is
complete & there are no
sequelae.
• Does not occur progression
to SJS-TEN.
• Recurrences are common.
STAPHYLOCOCCAL
SCALED SKIN
SYNDROME (SSSS)
DEFINISI

Merupakan suatu bentuk

penyakit kulit yang berat dan
disebabkan oleh eksotoksin
eksofoliatif yang dihasilkan S.
aureus fage grup II dan di tandai
oleh pembentukan bula dan
eksfoliasi yang genera lisata.
ETIOLOGI

Toksin eksfoliatif yang

dikeluarkan oleh stafilokok
gafe grup II.
MANIFESTASI KLINIK
• Menyerang anak berumur (5 tahun
tapi jarang orang dewasa)
• Terjadinya mendadak disertai
demam.
• Bula  lunak, besar, berisi cairan
 cepat pecah  lembaran kulit
yang luas terpisah, mengerat dan
terlepas  permukaan luka yang
merah muda dan basah.
• Tanda Nikolsky  
PEM. HISTOPATOLOGIS
Adanya pembentukan dataran
pemisah di epidermis atas,
distratum granulosum dan
pemisahan lapisan epidermis
oleh cairan edema yang
membentuk bula. Pada
epidermis dan dermis terdapat
rekasi radang ringan.
PENGOBATAN

• Antibiotika
• Pengobatan cairan yang tepat

Penderita akan sembuh dan kulit

pulih dalam 10 - 14 hari dari
permulaan eritem asal saja tidak
terjadi infeksi sekunder.
THANK YOU
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