doi: 10.1111/j.1346-8138.2007.00334.

x Journal of Dermatology 2007; 34: 573576

CASE REPORT
Blackwell Publishing Asia

Juvenile generalized pustular psoriasis

Ting XIAO, Bo LI, Chun-Di HE, Hong-Duo CHEN
Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China

ABSTRACT
Generalized pustular psoriasis (GPP) is an erythrodermic, generalized form of pustular psoriasis. GPP is rare in
children. The present study describes a case of juvenile GPP and reviews 12 juvenile GPP inpatients treated at
our hospital in the period 19782005.
Key words: cyclosporine, generalized pustular psoriasis, juvenile.

INTRODUCTION limbs, axillae and groin. Pustular pools could be seen

Generalized pustular psoriasis (GPP), or the
Zumbusch pattern of pustular psoriasis, is an eryth-
rodermic, generalized form of pustular psoriasis.1 It
is characterized by waves of widespread sheets of
sterile pustules on brightly erythematous skin, high
fever and general malaise. The occurrence of GPP in
childhood is rare, and fewer than 200 cases have
been reported in the published work.27 We herein
report an 8-year-old Chinese boy with juvenile GPP
and review 12 juvenile GPP inpatients treated at our
hospital in the period 19782005.
CASE REPORT
An 8-year-old boy presented with a 10-day history
of pustules and high fever. Itching and pain was
mild. His past history revealed that he had suffered
intermittent erythema and erosions on bilateral
axillae and groin 1.5 years prior. He had been diag-
nosed with eczema and treated with topical steroid
agents with some improvements. However, he received
no systemic medications. Moreover, he had no
previous personal or family history of psoriasis. The
pustules appeared on the neck first, then generalized
rapidly. Physical examinations revealed widespread
erythematous patches with a large number of pin-
point to milia-sized pustules on the flexural trunk,
on his abdomen (Fig. 1). A mung bean-sized ulcer
was seen on his palate.
Laboratory tests are summarized as: total white
blood cell count, 39.8 109/L with 90% segmented
neutrophils, 10% lymphocytes; erythrocyte sedimen-
tation rate, 34 mm/h; serum phosphate, 1.92 mmol/L
(range, 0.81.8); serum total protein 60 g/L (range,
6382); albumin, 29 g/L (range, 3550); serum calcium,
C-reactive protein, liver and renal function tests were
within normal ranges; repeated cultures from pustules
were all negative; and blood cultures were also negative.
Histological examination revealed a subcorneal
spongiform pustule filled with neutrophils. Perivascular
neutrophilic and lymphocytic infiltrations existed in
the superficial dermis (Fig. 2).
These clinicopathological findings confirmed the
diagnosis of juvenile GPP. p.o. cyclosporine 75 mg
twice daily, antibiotics, fluid and electrolytes were
given. The fever subsided within 4 days. The pustular
lesions resolved completely within 1 week (Fig. 3).
Two months later, the patient experienced a rebound
of pustular lesions but no fever because of discon-
tinuance of cyclosporin. The pustular lesions disap-
peared again after resuming administering cyclosporin
75 mg twice daily for a week. The patient remained
well with no new pustular lesions for 6 months and
is still in follow up. No obvious adverse effects have
yet been seen.

Correspondence: Professor Hong-Duo Chen, Department of Dermatology, No. 1 Hospital of China Medical University, 155 North Nanjing Street,
Shenyang, 110001, China. Email: hongduochen@hotmail.com or chenhd@cae.cn
Received 24 November 2006; accepted 13 February 2007.

2007 Japanese Dermatological Association 573

T. Xiao et al.
Figure 1. Widespread erythematous patches with a large
number of pinpoint to milia-sized pustules on the flexural
trunk, limbs and axillae. Pustular pools could be seen on
the patients abdomen.
Review of 12 juvenile GPP inpatients treated at
our hospital 19782005
Twelve children patients with GPP are described
(Table 1). The diagnosis of juvenile GPP was estab-
lished based on clinical features, histological features
and negative culture results of pustules. The 12
patients accounted for 0.992 of 12 095 inpatients
from 1959 to 2005. Of the 12 patients, eight were male
and four were female. The age at onset ranged
19 years (mean, 6.6 years). Interestingly, all of the
12 patients had no documented triggering factors.
Clinically, all of them had fever and generalized ster-
ile pustular lesions. None of them had lesions typical
of psoriasis vulgaris, family history of psoriasis, nor
did they suffer psoriasis vulgaris till now. Histologi-
cal examinations showed spongiform pustules in
the subcorneal layer of epidermis. Cultures of the
pustules for bacteria and fungi were all negative.
The total white blood cell count and erythrocyte
574
Figure 2. Subcorneal spongiform pustule filled with
neutrophils. Perivascular neutrophilic and lymphocytic
infiltrations existed in the superficial dermis (original
magnification (a) 100, (b) 400).
sedimentation rate was elevated in eight patients.
Serum phosphate levels were elevated in six patients.
However, serum calcium levels of all 12 patients
were within normal ranges. With regard to treatment,
systemic corticosteroid and antibiotics were given
to most patients. Tripterygium wilfordii Hook F
(an anti-inflammatory Chinese herbal medicine,
known in China as Lei-Gong-Teng) was used in three
patients together with corticosteroid. Methotrexate
was used in one patient together with corticosteroid.
Acitretin and corticosteroid were used in two
patients. Cyclosporine combined with corticosteroid
were used in two patients. Narrow-band ultraviolet
(UV)-B phototherapy and acitretin were used in one
patient. All of the patients improved and reached a
2007 Japanese Dermatological Association
 Juvenile generalized pustular psoriasis

that inheritance may play a role in the pathogenesis

of juvenile GPP.3,5,8 In addition, bacterial infection,
emotional stress and psychiatric morbidity have been
proposed as triggering factors of juvenile GPP.8,9
In a previous report by Ohkawara et al., two dis-
tinct groups of GPP were classified: (i) with a pre-
ceding history of ordinary psoriasis (pso+ GPP); and
(ii) without a psoriasis history (pso GPP).10 The
onset of pustular outbreak was earlier in the pso GPP.
The pso GPP group was more frequently affected
after infections, while the pso+ GPP group was
more frequently affected by preceding corticosteroid
therapy. The pso+ GPP group revealed a statistically
significant correlation with human leukocyte antigen
(HLA)-A1, HLA-B37 and HLA-DRw10, which was
closely related to psoriasis vulgaris patients in Japan.
According to their investigations, both clinical
surveillance and genetic analysis have disclosed
the heterogeneity of von Zumbusch type GPP. There
exist two types of GPP: (i) closely related to psoria-
sis; and (ii) not closely related to it.10 In the present
cohort, all 12 cases demonstrated typical clinical
features of juvenile GPP, such as characteristic
generalized sterile pustules, the coalescing of pus-
Figure 3. All pustules and erythematous patches tules into pus pools and the concomitant high fever.
disappeared 1 week after cyclosporine treatment. However, the absence of parakeratosis and pso-
riasiform hyperplasia in the histology of these cases
Table 1. Description of juvenile generalized pustular suggest that they are different from ordinary or
psoriasis (GPP) of 12 095 inpatients treated in our hospital classic psoriasis. There were no typical lesions of
1978 2005
psoriasis vulgaris seen during the acute phase or
Juvenile GPP the recovery phase. Also, none of the 12 patients
Total number 12 had familial history of psoriasis or other concomitant
% of inpatients 0.992 disease. It seems that all 12 juvenile GPP cases in
Age
Mean 6.6 years this study belong to the pso GPP group.
Sex With regard to differential diagnosis, impetigo
Female 4 (33.3%) herpetiformis (IH) is a candidate. IH was first reported
Male 8 (66.7%)
by von Hebra in 1872. Thereafter, there have been
reports on non-pregnant, male or childhood cases of
IH. IH has similar symptoms and histopathological
remission within 23 months. Recurrence was findings as GPP. Some investigators have insisted
common. Some patients recurred annually. Some that there is no reasonable evidence to suggest dif-
patients recurred several years later. ferentiating IH from GPP.1 Since then, in general, IH
has been defined as GPP in pregnancy or a pustular
form of psoriasis associated with pregnancy.11,12
DISCUSSION For treatment of GPP, a guideline has been
To date, the etiology of juvenile GPP is unknown. reported.13 Recently, there have also been some
There are several case reports about juvenile GPP successful case reports using cyclosporine, retinoids,
occurring in the same family or in siblings, indicating narrow-band UV-B phototherapy and dapsone for

2007 Japanese Dermatological Association 575

T. Xiao et al.
juvenile or childhood GPP.1416 Corticosteroid is an
effective choice for acute-stage or life-threatening
GPP patients, but recurrence and long-term adverse
effects are main concerns surrounding it. Cyclo-
sporine is one of the first-line systemic therapies for
GPP (the other three are etretinate, methotrexate
and glucocorticosteroids). Although the guidelines
for the treatment of psoriasis vulgaris with cyclo-
sporine have been formulated and proposed,
guidelines for GPP are not yet available. In the present
study, cyclosporine was used as a monotherapy. A
very quick and excellent clinical response was seen
in this case. Recurrence was not severe though
cyclosporine was stopped suddenly. Resuming
administration of cyclosporine was still effective.
Moreover, cyclosporine has less long-term adverse
effects than corticosteroid, retinoids, methotrexate
or dapsone in children. Because our study cohort
was only one case, further studies using larger
cohorts and long-term follow-up trials should be
carried out to define the role of cyclosporine in
juvenile GPP.
ACKNOWLEDGMENTS
We are grateful to Professor Kunihiko Tamaki
(Department of Dermatology, University of Tokyo) for
reviewing the manuscript.
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