Emergency In Dermatology

ACUTE BLISTERING
AND
EXFOLIATIVE SKIN
Nyoman Suryawati

Bagian/SMF Ilmu Kesehatan Kulit dan Kelamin FK

UNUD/ RSUP Sanglah Denpasar

Definition
1.

A serious situation or occurrence

that happens unexpectedly and
demands immediate action

2.

A condition of urgent need for

action or assistance : a state of
emergeny.

Some potential emergent dermatologic disease:

1.
2.

Stevens- Johnson Syndrome (SJS) and

Toxic Epidermal Necrolysis (TEN)
Staphylococcal Scalded Skin
Syndrome (SSSS)

Erythema Multiforme (EM)

An

acute self limited-disease, usually

mild,
and
often
relapsing
mucocutaneus syndrome
Related to an acute infection, most
often a recurrent Herpes Simplex
Virus (HSV) infection
Clinical characteristics : target-shaped
plaques predominant on the face and
extremities
Occurs in patients all ages, but mostly
in adolescences and young adult
There is a slight male preponderance 4

Erythema Multiforme (EM)

Erythema

multiforme subtype:
erythema multiforme minor :
skin lesions without involvement of mucous
membranes

erythema multiforme mayor :

skin lesions with involvement of mucous
membranes

herpes-associated erythema multiforme

mucosal erythema multiforme :
mucous membrane lesion without cutaneous
involvement
5

Erythema Multiforme (EM)..

Etiology

Infection :
pneumoniae)
Immunization
Drugs (rare)
Clinical

viral

(HSV),

bacterial

(M.

manifestation :

Prodromal
symptoms:
upper
respiratory
infection (cough, rhinitis, low grade fever)
skin rash occur in a symetric, acral distribution
on the extensor surfaces of the extremities
(hands and feet, elbows, and knees), face and
neck
First appear acrally and then spread in a
6
centripetal manner

Erythema Multiforme (EM)..

Typical target lesion consist of at least

3 concentric components:

a dusky central disk or blister

more peripherally, an infiltrated pale ring
an eryhtematous halo

An atypical target lesion consist of 2

rings
Mucous membrane lesion often
limited to oral cavity
Eye involvement begins with pain
and bilateral conjunctivitis

Erythema Multiforme.

Erythema Multiforme.

Typical target lesions on

the palm

Multiple concentric
vesicular rings (herpes
iris of Bateman)
9

Erythema Multiforme.

10

Erythema Multiforme.
The aim of treatment are to reduce
the duration of fever, eruption, and
hospitalization
M. pneumoniae infection :

antibiotics (macrolides in children,

macrolides or quinolone in adult)
Liquid

antacids, topical
glucocorticoids, local anesthetics relief
symptoms of painful mouth erosion

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Stevens- Johnson Syndrome

(SJS) and Toxic Epidermal
Necrolysis (TEN)
SJS

and TEN are acute life threatening

mucocutaneous reactions characterized by
extensive
necrosis
and
detachment
of
epidermis

These

2 conditions represent severity variant of

identical process that differs only in the
percentage of body surface involvement

Occurs

in patients all ages, with the risk

increasing with age after the fourth decade

More
The

frequently affects women

overal mortality is 20-25 % : SJS (5-12%),

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TEN (>30%)

SJS and TEN

Clinically

begins within 8 weeks (4-30 days)

after the onset of drug exposure

Non

specific symptoms : such as fever,

headache, rhinitis, myalgias may precede the
mucocutaneous lesion by 1-3 days

Pain

on swalloling and burning

the eyes

or stinging of

Course

is much more prolonged and severe

than erythema multiforme minor
a review stomatitis (100%), ocular
involvement (86%), genital mucosal or urethral
involvement (41%)

In

13

SJS and TEN

The

eruption is initially symetrically distributed

on the face, the upper trunk, and the proximal
extremities
The initial skin lesions : erythematous, dusky
red, purpuric macules, irregularly shaped,
which progressively coalesce
Confluent of necrotic lesions leads to extensive
and diffuse erythema
Nikolsky sign (dislodgement of the epidermis by
lateral pressure) : + on eryhtematous zone
The lesion evolve to flacid blister which spread
with pressure and break easily
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SJS and TEN

Patients

are classified according to the total of

area in which the epidermis is detached :
SJS : 10% of BSA
SJS/TEN overlap : 10-30%
TEN : > 30% BSA
Mucous membrane involvement (at least 2
site): 90% cases
It begins with erythema followed by painful
erosions of the bucal, ocular, and genital
mucosa
85% with conjuctival lesion : hyperemia,
erosions, chemosis, photophobia, lacrimation
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SJS and TEN

Etiopathogenesis: Cell mediated cytotoxic
reaction
The 4 etiologic categories are;
1. infectious
2. drug-induced
3. malignancy-related
4. idiopathic.
Infectious diseases that have been reported
include herpes simplex virus (HSV), influenza,
mumps, cat-scratch fever, mycoplasmal infection,
lymphogranuloma
venereum
(LGV),
histoplasmosis, and cholera.
In children, Epstein-Barr virus and enteroviruses
have been identified.

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SJS and TEN

Medication and the risk of epidermal
necrolysis
High risk :
Sulfamethoxazole, allopurinol, sulfadiazine,
sulfapyridine, sulfadoxine, sulfasalazine,
carbamazepine, lamotrigine, phenobarbital,
phenytoin, phenylbutazone, nevirapine, oxicam
NSAID, thiacetazone

Lower risk:
Acetic acid NSAID (diclofenac), aminopenicillins,
cephalosporins, quinolones, cyclins, macrolides
SJS is idiopathic in 25-50% of cases.

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SJS and TEN

Nikolsky sign

18

SJS and TEN

19

A. Erythema multiforme:
typical targets

C. Overlap Stevens-Johnson
syndrometoxic epidermal
necrolysis detachment of the
epidermis and erosions on 10%
to 29% of the body surface area.

B. Stevens-Johnson syndrome:
Confluence of individual
lesions remains limited,
involving less than 10% of the
body surface area.

D. Toxic epidermal necrolysis:

widespread detachment of
epidermis on more than 30% of
the body surface area.
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SJS and TEN

Patients with SJS and TEN then should be
treated with special attention to airway and
hemodynamic stability, fluid status,
wound/burn care, and pain
control
hospitalization
Treatment of SJS and TEN is primarily
supportive and symptomatic Fluid
Replacement
Sterile technique
Manage oral lesions with mouthwashes.
Areas of denuded skin must be covered with
compresses of saline solution.
Consultation eye , ENT, internal

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SJS and TEN

Underlying diseases and secondary infections
must be identified and treated.
Offending drugs must be stopped.
Treatment with systemic steroids
(MethylPrednisolon, Cortison, Dexametasone)
controversion
life saving drug

22

SJS and TEN

The performance of the score is at its best

on day 3 of hospitalization

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Staphyloccal Scalded Skin

Syndrome (SSSS).
Syn- Ritters disease or Pemphigus
neonatorum
Induced epidermolytic exotoxins (exfoliatin) A
and B, released by S. aureus and cause
detachment within the epidermal layer throgh
damage of desmosome
Desmosomes are the part of the skin cell
responsible for adhering to the adjacent skin
cell.
The toxins bind to a molecule within the
desmosome called
Desmoglein 1 and
break it up so the skin cells become gap intra24

SSSS.
The

syndrome begins by fever and diffuse

erythema.

Large

flaccid bullae with clear fluid form

and rupture almost immediately.
Characterised by red blistering skin that
looks like a burn or scald
SSSS

has no initial target lesions, the

erosions are more superficial and less
weepy, oropharyngeal lesions are rare
Skin biopsy is helpful
intraepidermal
separation
This
condition associated with high
mortality is seen commonly in infants and25

SSSS.

Nikolsky sign
gentle pressure to the skin of the arm
has sheared off the epidermis, which
folds like tissue paper.
26

SSSS.
Can

be differentiated from TEN by a

Tzanck test

In

SSSS, the blister cleavage plane is

intraepidermal
In TEN, separation is seen below the
basement membrane (in the upper
dermis)

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SSSS.
Requires

hospitalisation
intravenous antibiotics are generally
necessary to eradicate the
staphylococcal infection.
A penicillinase-resistant, antistaphylococcal antibiotic such as
flucloxacillin is used.
Depending

on response to treatment,
oral antibiotics can be substituted within
several days.
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SSSS.
Other

supportive treatments include:

Paracetamol when necessary for fever
and pain.

Maintaining
Skin

fluid and electrolyte intake.

care (the skin is often very fragile)

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SJS

20-40 yr
Etiology

Hypersensitivity
reaction (drug)
Clinical Feature Skin, > 2 mocous
membarane, target
lesion
10 % body surface
Bulla

Subepidermal

Patogenesis

Cell mediated
cytotoxic
reaction
10 %

Mortality
Therapy

Fluid n Electrolyte,
Corticosteroid

TEN

SSS

Uncommon in young
children
Hypersensitivity
reaction (drug)
Confluent morbiliform
eruption, blistering
skin exfoliation
>30 % body surface
Subepidermal

Common in infant
and children

Cell mediated
cytotoxic
reaction
20-40 % (severe
variant of SJS)
Fluid n Electrolyte,
Corticosteroid

Exotoxin Staphyloco.
aureus

Infection S. aureus

Red blistering skin,

scalded, look like
burn
Intraepidermal

<3%
Antibiotic

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Thank You

31

Learning Task
Case 1
A male, twenty years old, come to
emergency room Sanglah hospital with
itchy rash all over the body. There were
history of fever, malaise, sore throat 5
days before, and took some medicine
such as amoxicillin and paracetamol 2
days before the rash.
32

General

condition is weak, compos

mentis, good nutritional status, blood
pressure 120/80 mmHg, temperature
39C,
heart
rate
80x/minute,
respiration rate 20x/minute. From skin
examination we find purpuric lesion
and
multiple
bullae
on
the
erythematous skin with more than 30
% body surface area.

33

Learning Task
What other information should we ask
from anamnesis ?
What other physical examination we
should do?
What is the differential diagnosis for the
case?
Please explain what kind of laboratory
examination should we do to perform the
diagnosis?
What is the diagnosis of the case?
How should we manage the case?
What information should we give to the34

Self Assesment
Explain

the patomechanism of
the case
Discuss about complication of the
case
Explain the prognostic of the
case

35

Case II
A baby, 2 month old, come to
dermatology polyclinic, Sanglah hospital
with peel of skin on the neck since 2 days
ago.There were history of fever and
cough 4 days before and wound around
the nose since 1 day before the skin
problem. General condition
is weak,
good nutritional status, temperature
38,5C, heart rate 120x/minute and
respiration
rate
20x/minute.
Skin
examination from neck area, there were
eryhematous macule with ill defined
margine, some area with peel of skin.36

Learning Task
What

other information should we ask

from anamnesis ?
What other physical examination we
should do?
What is the differential diagnosis for the
case?
Please explain what kind of laboratory
examination should we do to perform
the diagnosis?
What is the diagnosis of the case?
How should we manage the case?
What information should we give to the37

Self Assesment
Explain

the patomechanism of
the case
Discuss about complication of the
case

38