A FATAL CASE OF ERYTHEMA MULTIFORME EXUDATIVUM

(STEVENS-JOHNSON'S SYNDROME) FOLLOWING THERAPY WITH

DILANTIN
John C. Watts
Pediatrics 1962;30;592-594

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it
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Copyright 1962 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005.
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A FATAL CASE OF ERYTHEMA

MULTIFORME
EXUDATIVUM
(STEVENS-JOHNSONS
SYNDROME)
FOLLOWING
THERAPY
WITH DILANTIN
John
Confederate

RYTH.EMA

and

known

1800s,

but

interest

uiated

by

Stevens

scnibed
it again
hypersensitivity
other

agents

in

and

Johnson,

herpes

sackie
tion

with
Ellis

all

B.C.G.
first

drome
dium

(Dilantin)

therapy

fatal.

with
The

this

case

drug.

J.,

mal

with

in

in this

2X-year-old

Negro
had
since

to better

dosage

later

he

advice

and

tivitis.

Penicillin

ADDRESS:

was

(J.C.W.)

be-

control

the

thereafter
His mother
told

that

he

in

injections
1400

South

inflammation

of age.

was

weeks

buccal

and

base.
some

mucosa,
same

Smith,

of

There
of the

were
bullae

present
on
edema
was
conjunctival

The

physical

tongue,

covered

white

in the

There

conhad

by a foul-ap-

were

numerous

present

of the

remarkable.

covered
gums

of his
lesions

exudate.
There
The lips were

greyish

were
was

with

surrounded

membrane.

and

type

there

mainder
not

partially

greyish

exudate

and
Fort

skin

bullous

with
purulent
involvement.

corneal

crusted
pearing

conjunc-

Street,

no

added

and

The

showed

of formation,

erythematous
lesions,
and

treated

by fever
and
sought
medical
given

significance

revealed
an acutely
with
temperature
of

rectally.

trunk

stages

of

ruptured.
Small
macules
were
the palms
and soles.
Perionbital
present
bilaterally,
and marked

the

were

various

of age,

manifestations

epilepsy.

Physical
examination
semicomatose
boy
and

General

for the electroencephalofindings

consistent

106.8#{176}F (41.5#{176}C)

5
and

19 months

notlling

of idiopathic

of a cold,

had

at

out;

been

normal.

convulsive

a diagnosis

neck,

had

during
tile
first
18
Following
the onset

seizures
the

carried

face,

convul-

Three

mal

except
showed

ill,

with

was

symptoms

he

vomiting,
before
ad-

delivery,

were

found,
gram
which

by an
fluent

male

of 5 mg/kg/day.

shortly
eyelids.

report

been

sodium

developed

followed
swollen

was

19 months

diphenyihydantom

case

weight

was

subse-

REPORT

convulsions,

In an attempt

pa-

of diphenylhydantoin
fatally.

phenobarbital

sions,

latter

preillness

a generalized

Gestation,

progress
been
good.

of

were

3 weeks

and

birth
period

and
had

studies

cases
have
foland 10 days9 of

described

CASE
A.

His

1943.6

The

the

he had

gm).

neonatal

of grand

syn-

after

Other
1 month,8

gan
after
3 weeks
therapy
and ended

grand

with

so-

therapy

recovered.
2 weeks,

therapy

in

hospital

patients

health
months

thenapy.1#{176}

syndrome

diphenythydration

quently
lowed

the
Cox-

Stevens-Johnson

the

to the

The

diphenyihydantoin

developed

of

ill with
high
fever,
on his face.
Shortly

lb 14 oz (2,665

vaccina-

irradiation

described

following

tient

and

followed

day

History

with

associated

also

and

a rash

ointment

third

convulsion.

meningococ-

been

It has

and

the

fac-

infections

influenza,

have

syndrome.

acutely

mission

aspirin,

organism,

simples,

virus

de-

By

was

bromides,

and

Louisiana

ophthalmic

scnibed.

stim-

as causative

barbiturates,
Vincents

cus,

the

who

penicillin,

antipyrine,

typhoid,

was

Shreveport,

tetracycline

in

disease

incriminated

cinchophen,

of

this

M.D.

Center,

in 1922. It is thought
to be a
state,
with many
drugs
and

phenophthalein,

tile

described

Sulfonamides,

tons.

Watts,

Medical

EXUDATIVUM

MULTIFORME

was

C.

Memorial

with

membrane,

bullae.

Copious

pharynx.

The

examination
was

no

re-

was

involvement

Arkansas.
Pmnucs,

October

592

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1962

ARTICLES
of tile

genital

or

Laboratory

penianal

hemoglobin

and

adequate.

4%

fluid

was

was

sterile.

normal.

Hospital

19 mg/100

ml;

from
normal.

blood

After

cultures

were

consisting
and

tibiotics,
transfusion

antipyretic
was

cul-

treatment

and

Fever
up
30 hours

mission

hydrocortisone

was

venously

in a dosage

of 40 mg/day.

this

the

fever

started
spread

began

the

trunk

neck,

and

and denuded,
leaving
similar
in appearance
lashes

partially

mained

clear.

improved

became

The

and

scrolesions
on
and

dermis.

The

ne-

seemed

his temperature
Shortly
thereafter

hos-

rose
he

eyelids
loss

was

a thin
The

by
branches

tenial.

The

to
de-

and
died
on
before
death
a
suctioned
from

the

extensively

involved

eyelashes.

exudate

There

in tile

night

eye.

bronchus

was

oc-

aspirated
contained

vomitus,
more

and
of this

the
ma-

mucosa
and
the

slightly

to be

de-

a segmental

In the

remaining

widely

was

no
bullous
lingula
there

reddish-purple,

of atelectasis.

type

lobes

scattered

there

areas

bronchopneumonia

which

on

micro-

examination.

The

esophagus

grossly,

but

findings

was

the

There

area

ulcers

and

were

showed

no

not

except

for slight
system.

The

thought

the

to

be

meninges

brain
findings

of the

entire

was

1.5

and

cm

yenas
cere-

in thickness.

kidneys

spleen

the

peptic.

type
and

the

in

and

pathologic

dilation

in

as acute

This
was
interpreted
of hydrocephalus.
The

heart

liver

and

described

signfficant

tricular

an internal
bral cortex

stomach

were
of

esopha-

ulcerations

of the

Examination

showed

ulcerative

small

These

to be normal
sections

of acute
were

prepylonic

thought

histologic

typical

gitis.

the

skin

for

inflammation.

and

representing

small,

the

main-stem

deep

proved

of

acute

but
intact,
found.
Over

area

were

of

very mmremain-

except

tracheobronchial

edematous
lesions
were
pressed

vessels

were

left

and

in the

normal

of epidermis

eluded
distal

was

be

purulent

skin

to be absent
with a
debris
in its place.

blood

to

Mi-

involved

response

changes

with

the

hyperemia

The

thought

duodenum.

areas
eye-

ninth

slight

nonspecific

the
skin
and

corneas

to the

veloped
labored
respirations
the tenth
hospital
day. Just
large
amount
of mucus
was
mouth

and

condition

sixth

ing

scopic

confluent

The

childs

the

pital
day,
when
103#{176}
F (39.4#{176}C).

intraFollow-

large
weeping
to a burn.
The

sloughed.

from

104#{176}
F
after
ad-

orally.
The
extremities,

day his penis

The
earlier

face,

to

started

on the fifth hospital

tum
were
involved.

an-

A blood

subsiding,

taking
liquids
to the lower

was

fluids,

measures.

given.

continued,

(40.0#{176}C)

taken,

of intravenous

was

inflammatory

were

staph-

Course

started,

his

The
erytho-

A coagulase-positive

ens and
Conynebacterium
tunes.
A chest
x-ray
was

ing

There

was

tivae,
a
phanynx,

cilild
lesions

11%

imal

of infection.

of

cells,

cultured
from
the conjuncNeissenia
species
from
the
nasoand
alpha-hemolytic
streptococ-

ylococcus

no evidence

was

examination

revealed
the epidermis
thin layer
of necrotic

40

protein,

there

croscopic

79%

gm/100

Platelets

contained

0 leukocytes;

with

12.4

monocytes.

Urinalysis

cerebrospinal
and

of

41,00O/mm,
cells, 6% band

count,

lymphocytes,

cytes;

disclosed

concentration

polymorphonuclear
were

tion

regions.

examinations

1111; leukocyte

593

were

normal

showed

only

and
a mild

congestion.

pharynx.
COMMENT

Autopsy
The

Findings
autopsy

was

performed

over

Galloway.
pen chest,

The

skin

and

denuded.

Some

back
was almost
intact
bullous

present

the palms
contained

by Dr.

the head,

Edgar

neck,

up-

completely
lesions
were

distal
extremities,
including
soles. These were brown
and
a serous fluid. On gross examina-

over

the

and

Although
syndrome
usually
10

been

patients
are

recover.

reported

with

acutely

and

Until
cases

in

1954
which

Stevens-Johnson
severely
there

ill,
were

autopsies

they
only
had

performed.bO

The mortality
has been
estimated
at 5 to 18% in this disease.l2
The presence
or absence
of pulmonary
involvement
seems
to be most important
in

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594

ERYTHEMA

determining
years

the

there

OtiS

eye

prognosis.

has

been

In

probably

residuals,

the

a decrease

MULTIFORME

last

in the

due

to the

1.

probably

helped

to reduce

the

Scott,

T.

2,

Hypersensitivity

N.

E.,

Amer.,

and

syndrome,

1959.
3. Yaffee,

ity.

M. :

Clin.

Wasserman,
son

mortal-

F.

Pediat.

use

of antimicrobials.
The emphasis
on vigorous
supportive
treatment
and the use of steroids
ilas

REFERENCES

15
seri-

Class,

Arch.

H. : Erythema
B5. Arch.
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body,13

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In

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skin

It

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have been
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A fatal

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this

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4. Alvsaker,
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there

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one

the
skin

primarily
5110w

tile

htillous

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of

A FATAL CASE OF ERYTHEMA MULTIFORME EXUDATIVUM

(STEVENS-JOHNSON'S SYNDROME) FOLLOWING THERAPY WITH
DILANTIN
John C. Watts
Pediatrics 1962;30;592-594
Updated Information
& Services

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