Two Cases of Generalized Pustular Psoriasis Induced by Topical Steroids

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Acta Dermatologica Volume 77

(
3
), 197
-
200, 1983





197


Two Cases
of

Generalized

Pustular Psoriasis Induced
b
y Topical
Steroids

Sayoko Ohno, Yoshiki Miyachi, Motoaki Ozaki and Sadao Imamura

Department of dermatology, Faculty of Medicine Kyoto University,
Kyoto

[
Director:

Prof. S. Imamura
]

(
Received: January 6, 1982)



The first record of
generalized

pustular
psoriasis (GPP
) was made by Zumbusch in 1910
1)
,
noting

that it is a
disease

with
se
rious

systemic conditions accompanied with poor prognosis
.
In many
cases
,

G
PP

develops

from
psoriasis vulgaris
,

and

the greatest focus has been on systemic steroid
administration inducing it

2)
.

It is believed

that efficacious therapies are methotrexate (
MTX
)
,
folate antagonists such as hydroxyurea
, and PUVA therapy.
There have been no systematic
reports on the prognosis of G
P
P
;
however, it has been said that many cases tend to be relatively
prolonged.
T
he present paper will report on t
w
o G
PP

cases consid
ered to be induced by the use of
topical steroids during the progress of
psoriasis vulgaris
.


Case
s

Case
1:
A 19
-
year
-
old male student

Chief complaints
:
Fever and systemic
pustules

Familial

history: No psoriasis, and nothing else of note.

Past history: No
thing of note
.

Current history: From October 1980,

erythema

and
pustules

accompanying scales appeared on the
outside of the bilateral elbows, and similar
erythema

started appearing on the shoulders and
elbows
from Aril 1981.
The patient visited our
d
epartment and was diagnosed with
psoriasis
vulgaris
, and
simple topical administration of at least
10g per day of
clobetasol propionate

ointment was administered.
R
a
sh repeatedly appeared and disappeared; however, the patient
started
experiencing
chills in

late June, and small pustules with erythema started appearing in the
inferior abdominal part and thighs together with mild fever
.
Despite the topical administration of
clobetasol propionate

ointment
, symptoms gradually expanded to the entire body, and the
refore
the patient was admitted to our Department on July 28. No oral administration of steroids was
given.


Current history at the time of
hospitalization
: Body
temperature
: 37.6 °
C
.
Irregular
erythema

with
diameter
s

of
1 to 10 cm and with clear borders

a
ppeared throughout almost the entire body
except for the soles of the feet
, and small
pustules were observed

on
the

erythema

on the body
trunk

(Figure 1).
Ring
-
shaped
erythema

was partially observed.

Yellow
scabs
were firmly adhered
to the head
.

The nail
on the fourth finger o
n

the left

hand became rough and thick.

Geographic
tongue was observed (Figure 2)
.



198






Acta Dermatologica
Volume 77,
I
ssue
3


Figure 1
.

Small pustules in groups
a
re
present
on the erythema observed on the anterior chest,
and

some are in in a ring shape (C
ase
1)







Figure 2
.

Geographic tongue (Case 1)



Figure 3.Typical
s
ub
-
corneal spongiform pustules

(HE) (Case 1)

Figure 5.
Small pustules in groups on the
erythema observed on the back

(Case 2)


There were
no
swollen tonsils, red pharynx, hepatosplenomegaly,
nor

lymph node swelling
.

Examination findings at the time of
hospitalization
: elevated white blood
cell
count

(11800/

mm
3
),

mildly elevated sedimentation rate (27 mm/h), CRP (
+
), ASO value 320 times
.

Histol
ogical findings:

Biopsy was performed from the area of pustules
.
Spongiform pustules were
present under the epithelial corneum, and epidermal
ridges
were extended under the pustules
(Figure 3).

Treatment and progress (Figure 4)
:


Erythema

expanded even aft
er
hospitalization
, and pustules
covered almost the entire body. Fever became prominent, and the patient was suffering from pain
in the elbow joints. Due to this, 2.5 mg of methotrexate (MTX) was administered three times every
12 hours for one week, which
was one course of therapy. Topical
administration
of a mixture of
fluocinolone acetonide
ointment (up to 20 g per day)
,

zinc borate oxide ointment,
and
white
Vaseline

was
administered

for pain in the r
a
sh region.

The dosage of

fluocinolone acetonide
ointment

was gradually decreased as pain improved, and the topical steroid

was discontinued
after
one
month
.
Rash started improving
and fever disappeared
after completion of the third
course of MTX
.

No new rash appeared from late August, and since then, MT
X administration was
continued for a subsequent seven weeks, and the patient was discharged on September 10. At the
time of discharge, only a small number of pustules were observed on the palms. After discharge,
the patient was made to administer MTX at an

interval of once every two weeks, and
administration was discontinued
in late November
after a total of 10 administrations. Thereafter,
no relapse of pustules was observed, and

the entire
erythematic

rash

of
psoriasis

disappeared
.

Short
-
term exposure for
MPD
measurement
induced pustules, therefore, PUVA therapy was not
provided.

Case 2:
A
10
-
year
-
old female

Chief complaint: Small pustules in groups on erythema

Familia
l

history:

Elder

sister suffering from atopic dermatitis

Past history:

A
topic dermatitis

from the time when the patient was an infant

Current history:

In
June 1978
, small
erythema

appeared on the left abdominal part with no
particular cause, and scales increased on the head skin. The patient visited our Department on
September 2, and was diagnosed with
psoriasis vulgaris
.

The

patient was instructed to perform
simple application of

fluocinolone acetonide

cream (3g per day).
Approximately 2 weeks
thereafter, a large number of small particles started appearing on erythema
,
and they expanded
throughout the entire body
,

including

the palms, within several days.



August 1982








1
9
9


Hospitalization







D
ischarge




Progress and treatment (Case
2
)


Hospitalization










D
ischarge


Figure
4.
Small pustules in groups on
erythema

observed on the back
(Case
2
)


Since the patient started suffering from fever and systemic

malaise, she was
hospitalized

at our
Department on October 4
.

Current history at the time of
hospitalization
: Body temperature 38.8
°
C.
Showing apathetic f
acial
expression.

Small
pustules

were observed in groups on
erythema,
some of which were in ring
-
shape
s

on
the

skin of
the
limbs and the body trunk (Figure 5).
The head skin was covered with
thick

sc
a
b
s.


Mild pain

corresponding to rash was observed.

Pustules

on the
i
ntrabuccal
che
e
k
mucosa and geographic tongue were observed.

Mild redness and swelling o
f

the pharynx
was
observed
.
No
hepatosplenomegaly

nor joint inflammation were observed. Swollen lymph nodes
,

Topical

fluocinolone acetonide

Rash

Fever

PUVA therapy (17 times in total)


Rash

Fever

elastic upon palpitation
, and with
the siz
e of soya beans

were observed
at
several sites in the
cervical region and mandibular

part.

Examination find
ing
s at the time of
hospitalization
:

Elevated white blood cell count (13200/
mm
3
),

elevated sedimentation rate (
48

mm/h), CRP (
+
++
),
mild A
l
-
P
elevation (200 U)
.
No abnormalities
found on ECG, chest x
-
ray, and urine examinations. Bacterial culture from
pustules

was negative.

Histological findings:

Pustules

were found to be

Kogoj

s
subcutaneous spongiform
pustules

Treatment and progress (Figure 4)
:
S
ince fever,
pain in the rash part, and systemic malaise
continued, 1.25 mg MTX was administered
from early October

three times every 12 hours for one
week, which was one course of therapy.

Since the rash expanded during the administration period
,
PUVA t
herapy was commenced from
early October.
I
n early December
, s
ystemic symptoms
went
away
, erythema

and

pustules
disappeared
, and
no
new
pustules were observed
.
The
administration of MTX was discontinued after the completion of a total of nine courses, and t
he
patient was discharged on December 26.

No further treatment was provided after discharge;
however, relapse of pustules has not been observed

and psoriasis rash disappeared.

Discussion

The chief complaints in the present cases

were f
ever,

general
pustul
es

and
erythematic

rash.
From
a
clinical
perspective, the differential diagnosis may be one of
generalized

pustular
psoriasis

(GPP
),
impetigo herpetiformis
,

dissemination
-
type
Hallopeau

acrodermatitis continua
,
subcutaneous
pustulosis,
or other conditions.


The present cases were diagnosed with G
P
P based on the following facts
: (1)
the condition
developed during treatment for
psoriasis vulgaris
, (2)
there was no relationship with pregnancy or
parathyroid dysfunction
, (3)
t
here was no prominent local rash

at

the edges of limbs,
and
(4)
the
patients had subjective and systemic symptoms
.

The first report on
GPP

was made in 1910 by

Zu
mbus
c
h

1)
.
Its clinical ch
aracteristics are chills,
fever

accompanied with

widespread erythema

and
a large number of pustules
,
and
desquamation

with the appearance of scarlet fever
;

and
such a series of changes repeat every few days or few
weeks
. Baker et al.
3)

categorized GPP into the following four types based on its clinical findings: (1)
Zumbusch type, (2)
a
n
nu
l
ar

type
,
(
3
)
e
xanthematic
type,
and
(4)
localized

type. Since the present
two cases presented

with
rash, systemic conditions and re
flaring,
both of them were considered to
be

the
Zumbusch type
.

The c
ause
s

of progression to pustules

are
arsenical

agents,
antimalarial agents, and stimulation
through local therapy
4
)
, infectious diseases, stress
5)
, sunlight irradiation
,
and
endocrine disorder
.
From amongst these causes, the
greatest focus has been on the systemic administration of
steroids
.
T
here has also b
een

a

report stating that
the
increasing frequency of G
P
P

in recent years
is caused by steroids

2)
.

Furthermore,
Hellgren
7)

and Boxley
8
)

recently reported that a strong
steroid topical agent

caused progression of psoriasis
to include
pustules
.



200






Acta Dermatologica Volume 77,
I
ssue
3


Cases reported by

Hellgren

receive
d

a single application of 100 g of
betamethasone diprpionate
ointment

on
consecutive days
,
and were observed to develop psoriasis into pustules in 11 to 22
days thereafter. Also,
the
Boxley et al
.

case
was observed to be suffering from pustules developed
from psoriasis

due to sudden discontinuation of

a
single
application

of a large dosage of
clobestasol propionate ointment. Kondo et al.
9)

found that patients suffering from psoria
sis
developed pustules

after one

and a

half years of simple application of steroids
.
Systemic simple
application of
f
airly strong steroid ointment

was given to the present cases for 4 to 8 months,
although the dosage was not as large as those in in Hel
lgre
n et al.

s
cases
,
and it is considered that
this application induced progression to
p
ustules
.
Case
1
was observed to have elevated ASO values,
but was not observed to have any abnormalities in parts such as the pharynx
,
therefore, the
possibility of manife
st infection with
hemolytic streptococcus
was ruled out.

Thus
it is considered
less likely that the present case was
one of
G
P
P induced by infection.

In treatment, Case
1
in the present study

was
made to administer

fluocinolone acetonide
ointment

at the
be
ginning
;
however, the
administration
was discontinued
mid
way through, and
only the oral administration of MTX was continued.
C
ase
2
underwent concomitant therapy with
oral administ
ration of

MTX

and PUVA therapy.

Neither of the cases orally administered steroids.

The oral administration of steroids can be a therapy for
GPP
; however, there are many cases
10)

which do not respond to administration or in which
symptoms

further deteriorate following
discontinuation
,

e
ven though symptoms improve

at one point; therefore, it is believed that the
oral administration of steroids should be avoided as much as possible
.
In
the
cases we have
experienced,
topical agent
s

such as
zinc borate oxide ointment

were efficacious for loc
al pain.
Therefore, unless it is unavoidable,

oral
administration of steroids

should not be adopted and
symptomatic treatment should be provided for
symptoms
such as pain
5)
. O
o
taki et al.

2)
, Kondo et
al.
9)

and Miyaji et al.
11)

also insist that topical
steroid

agent
s

should be avoided as much as
possible.

According to Ryan and Baker
12
)

it is relatively easy to treat cases which start suffering from
GPP

induced by some cause after long
-
term suffering from typical
psoriatic

rash which starts while the
patient is young,
whereas
it is difficult to treat cases
who start suffering from a typical

psoriatic

rash

in
or
after middle age which develops to
G
PP in a short period of time
,
and
in such cases
it
may become life
-
threatening
.

Both of the two cases which we experienced started suffering from
psoriatic

rash

while they were young and quickly developed into
GPP

in

a short
period of time;
however,

the prognoses were extremely good.

Although the observation period was short, the
fa
ct that not only pustules but also

psoriatic

rash

disappeared should be focused

on.


Conclusion

The present paper reported t
w
o cases of
G
PP which were

considered to have been induced by the
simple application of topical steroids
.
Both of the two cases
started suffering from psoriatic rash
while the
y

were young, which developed into
GPP

in a short
period of time.

The oral
administration of steroids was not adopted in treatment,
the oral
administration of
MTX

and
PUVA therapy

were adopted, and progress w
as followed up.

Topical
steroid

steroids were
also
avoided as much as possible.

Treatment effects were
favorable
, systemic symptoms such as fever
and systemic malaise improved at the same time as pustules disappeared
, and
psoriatic

rash

has
not relapsed to

date.


Details of Case 2

are reported in Reference 11.




References


2) Noriko Ootaki et al.
Journal of
Clinical
D
ermatology, 17:75, 1975.


9)
Koju Kondo et al.:
Clinical
D
ermatology, 35:

2
2
5, 1981.


10)
Moshimasa Shimada
et al.
:
Clinical
D
ermatology,

30:

975, 1976.

11)
Yoshiki Miyaji
et al.
:
Clinical
D
ermatology,

34:

395, 1980.