KEGAWAT KEGAWAT DARURATAN DARURATAN

KULITKULIT

ERYTHEMA ERYTHEMA MULTIFORMEMULTIFORME

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.

E t i o l o g y

• Triggered by HSV-1 & HSV-2.

• Drugs as rare cause of EM.

P a t h o g e n e s i s

• 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.

P a t h o l o g y

• 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 & frank

epidermal necrosis.

Differential Diagnosis

• Acute annular urticaria.

• Urticaria vasculitis.

• Disseminated lesion of contact dermatitis.

• Bullous pemphigoid.

• Linear IgA dermatosis.

• Herpes gestationes.

T r e a t m e n t

• 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.

P r o g n o s i s

• Self limited, recovery is

complete & there are no

sequelae.

• Does not occur progression

to SJS-TEN.

• Recurrences are common.

STEVENS-JOHNSON STEVENS-JOHNSON SYNDROMESYNDROME

STEVENS-JOHNSON STEVENS-JOHNSON SYNDROMESYNDROME

Episodic acute mucocutaneous

intolerance reactions most

often elicited by drugs & less

so by infections..

> 50% drug

Minority infections,

vaccination or graft-versus-

host (GVHD)

Etiology

Pathogenesis

Hypersensitivity reaction

III & IV type..

Trias sign :

1. Skin.

2. Mucous-membrane.

3. Eye.

Clinical Feature

It begins nonspecific prodrome :

• Fever • Malaise

• Headache • Rhinitis

• Cough • Sore throat

• Chest pain • Vomiting

• Diarrhea • Myalgia

• Arthralgia.

Skin :

• Erythematous (sometimes

morbiliform rash).

• Vesicle.

• Bullous, pustuler 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.

• Satellite-cell necrosis (early

stages) epidermal

eosinophilic necrosis of the

basal & suprabasal layers

subepidermal separation.

Histopathology

• 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.

• SSSS staphylococcal

epidermolisyn toxynemia

subcorneal acantholysis.

• Macular drug eruption.

• Fixed drug eruption.

• Acute GVHS.

• Viral exanthems.

Complication

Toxicity, dehydration, water &

electrolyte imbalance

hemodynamic shock.

Pulmonary edema, mental

obtusion, confusion, coma &

seizure..

• Skin of heal : hyper and/ or

hypopigmentation.

• Mucosa : scarring.

• Eye :

Symblepharon, synechiae

corneal opacities or

scarring blindness..

Late Complication

• 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..

Treatment

• 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..

• KCl 3 x 500 mg/d K level .

• Monitoring Hm, 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..

Mortality rate severity

disease & medical care.

Prognosis

TOXIC EPIDERMAL TOXIC EPIDERMAL NECROLYSIS NECROLYSIS

(TEN)(TEN)

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.

DEFINITION

Drugs (the leading causative

factors).

Infection.

Vaccination.

Graft versus host disease

(GVHD).

ETIOLOGY

It is a polyetiologic reason pattern :

PATHOGENESIS

Cytotoxic immune reaction Cell T (CD4

& % CD8)

TNF α

Inducing apoptosis

destruction of epidermis & keratinocyte

TEN begins with a

nonspecific prodrome of 1

to 14 days in at least half of

patients.

CLINICAL FEATURES

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 :

Constitutional sign.

Internal organ involvement.

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.

Erythema multiforme with

extensive eosinophilic necrosis

of the epidermis & cleavage

plane above the basement

membrane.

HISTOPATHOLOGY

An elevated blood

sedimentation rate.

Moderate leukocytosis,

anemia.

LABORATORY INVESTIGATIONS

Fluid-electrolyte imbalances,

microalbuminuria, hypo-

proteinemia.

A transient decrease of

peripheral CD4+ T lymphocyte

counts.

Tzank preparations showing

cuboidal cells & skin biopsy can

be used to confirm the

diagnosis.

DIAGNOSIS

Staphylococcal scalded skin

syndrome.

Generalized fixed drug

eruption.

Burns, cauterizations, etc.

Toxic erythroderma.

DIFFERENTIAL DIAGNOSIS

Systemic glucocorticoids 80

to 120 mg of

methylprednisolone per day by

mouth until disease

progression has ceased.

TREATMENT

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.

Severe morbidity & high

mortality (20-30%).

Death is usually due to :

Sepsis.

Gastrointestinal hemorrhage.

Renal, hepatic or pulmonary

complications.

PROGNOSIS