[Downloaded free from http://www.jdrntruhs.org on Thursday, January 10, 2019, IP: 190.233.49.

41]

Original Article

Pustular psoriasis – Clinical study in a tertiary care

center
Sudha Rani Chintagunta, Sudha Vani Damarla1, Prema Jyothi Gopidi1, Geetakiran Arakkal1
Department of DVL, Government Medical College, Nizamabad, 1Deparment of DVL, Gandhi Medical College and
Hospital, Hyderabad, Telangana, India

ABSTRACT
Background: Pustular psoriasis is an uncommon form of psoriasis consisting of sheets of pustules on erythematous background.
Treatment depends on the extent of involvement, severity, and underlying risk factors.
Objectives: To study the clinical profile of pustular psoriasis, to identify triggering factors, and to study the response to treatment.
Materials and Methods: A prospective clinical and therapeutic study of patients with pustular psoriasis attending DVL department
over a period of 2 years. Diagnosis was made based on clinical and histopathological findings. Fourteen patients were admitted,
investigated, and treated appropriately.
Results: Nine were females and five were males. The mean age of the patients was 30 years. The duration of disease was 1–3 weeks.
Among 14 cases of generalized pustular psoriasis, 10 were of acute type, 3 were pregnancy‑related, and 1 childhood onset. In pregnancy
group, all the patients were managed with systemic steroids till delivery followed by methotrexate (Mtx) and cyclosporine (CsA) during
postpartum period in two patients, respectively. Eight patients were managed with short course of steroids and Mtx followed by
Mtx alone. One patient with tuberculosis spine was managed with CsA and antituberculous therapy. Highly active retroviral therapy
and acitretin (50 mg) were given in a retroviral‑positive patient. One patient with recurrent episodes improved with antibiotics only.
In our study, steroids were followed by maintenance therapy with Mtx/CsA and acitretin accordingly. CsA was given during crisis
followed by maintenance with acitretin. Two patients on Mtx and one on CsA therapy with frequent flares were shifted to acitretin.
Acitretin showed good response in patients who had frequent flares while on Mtx and CsA. In our study, response with acitretin,
Mtx, and CsA was 100%, 57.1%, and 50%, respectively.
Limitations: Only small number of patients.
Conclusion: All patients were generalized type and showed good remission except one who succumbed to death due to septicemia.

Key words: Clinical profile, pustular psoriasis, therapeutic response

INTRODUCTION other forms of psoriasis. The course of generalized

pustular psoriasis (GPP) varies from acute, subacute,
Pustular psoriasis is an uncommon form of psoriasis or chronic. The acute type (von Zumbusch variant)
consisting of sheets of pustules on erythematous is associated with constitutional signs and symptoms
background. It can occur de novo or progress from and systemic complications. The common subacute
and chronic types present with skin symptoms only.
Address for correspondence: There are limited data in literature about guidelines
Dr. Sudha Rani Chintagunta, Plot No. 5, Jupiter Colony,
Kakaguda, Kharkhana, Secunderabad, Telangana, India.
for the therapy of pustular psoriasis. The treatment
E‑mail: schintagunta@gmail.com
This is an open access journal, and articles are distributed under the
terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike
4.0 License, which allows others to remix, tweak, and build upon the
Access this article online
work non‑commercially, as long as appropriate credit is given and
Quick Response Code:
Website: the new creations are licensed under the identical terms.
www.jdrntruhs.org For reprints contact: reprints@medknow.com

DOI: How to cite this article: Chintagunta SR, Damarla SV, Gopidi PJ,
Arakkal G. Pustular psoriasis – Clinical study in a tertiary care center. J
10.4103/JDRNTRUHS.JDRNTRUHS_109_17
NTR Univ Health Sci 2018;7:259-64.

© 2019 Journal of Dr. NTR University of Health Sciences | Published by Wolters Kluwer - Medknow 259
[Downloaded free from http://www.jdrntruhs.org on Thursday, January 10, 2019, IP: 190.233.49.41]
Chintagunta, et al.: Pustular psoriasis
depends on the extent of involvement, disease
severity, age, and underlying risk factors. [1] We
studied 14  patients for the clinical profile, triggering
factors, and response to treatment. Based on severity,
associated risk factors, and availability of drugs in
our institute, we started the patients on systemic
steroids, methotrexate (Mtx), cyclosporine (CsA), and
acitretin. Although retinoids are the drug of choice,[2‑4]
Mtx was used in a majority of our patients due to
nonavailability of retinoids in the institute.
MATERIALS AND METHODS
The study was conducted from November 2014 to
December 2016 at a tertiary care center for a period
of 2 years. It was a prospective observational study
of 14 patients. There is a separate psoriasis clinic in
our institute, where during a 15‑year period we hardly
came across pustular psoriasis. Only two cases were
recorded. Recently, many cases were encountered in
a short period which were referrals from peripheral
centers. Of 14 cases, only 2 cases are institute cases
who were attending psoriasis clinic and 12 cases were
referrals. The objectives were to study the clinical
profile of pustular psoriasis, to identify triggering
factors, and to know the therapeutic response.
Fourteen cases of pustular psoriasis were included; all
of them were generalized type. All the cases presented
with generalized erythema, sheets of pustules, and
systemic symptoms, meeting the diagnostic criteria for
GPP as suggested by Umezawa et al.[2] Diagnosis was
made based on clinical and histopathological findings.
Nine cases were known chronic plaque psoriasis and
five presented de novo with pustular psoriasis without
previous history of psoriasis. All cases were clinically
examined, admitted, and investigated. Complete blood
picture (CBP), Complete urine examination (CUE),
Random blood sugar (RBS), Liver function tests
(LFT), Renal function tests (RFT), lipid profile, HIV,
HBsAg, Hepatitis C virus (HCV), serum calcium,
serum electrolytes, serum proteins, electrocardiogram,
X‑ray chest, ultrasound abdomen, blood culture,
C‑reactive protein, pus for culture and sensitivity,
Anti streptolysin O (ASO) titer, Gram stain, and skin
biopsy were done. Patients were started on Mtx, CsA,
and acitretin depending on the age, severity, and risk
factors.
OBSERVATIONS AND RESULTS
Of 14 patients, 9 were females and 5 were males.
260 Journal of Dr. NTR University of Health Sciences | Volume 7 | Issue 4 | October-December 2018
Male‑to‑female ratio was 1:1.8. The age ranged
between 9 and 55 years with a mean age of
30 years. The duration of disease varied from 1
to 3 weeks. Among 14 cases of GPP, 10 were of
acute type with adult onset, 1 childhood GPP, and
3 were pregnancy‑related. Geographic tongue was
observed in two patients [Figure 1]. Precipitating
factors and associated conditions are mentioned
in Table 1. Nine patients were acutely toxic
without any evidence of septicemia. In pregnancy
group, two patients presented at 28 and 32 weeks
gestation, respectively, and one patient at 12 weeks.
Two patients who presented at 28 and 32 weeks
were managed with systemic steroids (1 mg/kg/
day) till delivery with good maternal and fetal
outcome [Figure 2]. There was no remission in
postpartum and both were not lactating. Mtx (7.5 mg)
in one and steroid in combination with CsA 3mg/
kg in another was given for 6 weeks. The case of
GPP which started at 32 weeks of pregnancy was
treated outside the institute with systemic steroids.
She presented to us during postpartum and she
was on 60 mg prednisolone with dependency and
cushingoid features. As the condition is severe and
the patient is toxic, we ruled out septicemia and
started on CsA with tapering of steroids. In both the
cases, response was not satisfactory and there was
continuation of new lesions. Both the cases showed
complete remission with 0.75 mg/kg (25, 50 mg) of
acitretin. Both the patients developed inflammatory
changes at the nail folds [Figure 3], which was not
dose‑dependent and managed with lowering of the
dose and topical steroid and antibiotic combination.
The third patient who presented at 12 weeks was
managed with 40 mg prednisolone with tapering dose
and the patient was aborted subsequently.
One patient was a known case of Pott’s spine
and on antituberculous therapy was managed with
CsA. One patient who was positive for retroviral
disease with CD4 count 450 on highly active
antiretroviral therapy (HAART) presented with GPP.
In addition, the patient was started on 50 mg of
acitretin. Complete remission occurred in 12 weeks.
Acetretin gradually tapered and HAART therapy was
continued [Table 2].
Mtx 7.5–10 mg weekly along with folic acid was
started in eight patients (five patients on Mtx and
prednisolone and only Mtx in three) [Figure 4].
Early response was observed in 2–4 weeks and
[Downloaded free from http://www.jdrntruhs.org on Thursday, January 10, 2019, IP: 190.233.49.41]
Chintagunta, et al.: Pustular psoriasis
Figure 1: Acute generalized pustular psoriasis with geographic
tongue
Figure 3: Nail fold inflammation and ulceration following acitretin
complete remission in 4–6 months [Table 2]. The
patient with h/o of recurrent episodes was managed
with only antibiotics (amoxycillin and cloxacillin)
who is a known case of plaque psoriasis of 15 years
duration. He was on Mtx on and off. Since 2 years
he is presenting with GPP picture, subacute in nature,
which responded well with admission and antibiotics
followed by symptomatic treatment. Treatment details
are summarized in Table 2.
DISCUSSION
The age group among adult patients with pustular
psoriasis is reported to be between 21 and 81 years
with a mean age of 40.9 years.[5] In our study, the
common age group affected was 9–55 years with an
average age of 30 years. Male‑to‑female ratio was
1:2 with female predominance as reported in the
literature.[5,6] All the patients were of GPP type and no
localized variant was observed. The majority (71%)
of the patients belong to acute or von Zumbusch type
of GPP.
Journal of Dr. NTR University of Health Sciences | Volume 7 | Issue 4 | October-December 2018
Figure 2: Generalized pustular psoriasis of pregnancy, response
to systemic steroids
Figure 4: GPP response to systemic steroids and methotrexate
Management of GPP still remains a major challenge;
acitretin, Mtx, CsA, and infliximab are considered
first‑line therapies by the National Psoriasis
Foundation Medical Board.[1] Systemic steroids are
used in the acute stage[2] and antipsoriatic treatment is
started simultaneously. The treatment of choice during
pregnancy and lactation is systemic corticosteroids,
with 30–60 mg of prednisone per day.[2] Cyclosporin
may be used in refractory cases.[7‑9]
In our study, therapeutic choice was chosen based
on severity, comorbid conditions (TB, HIV), and
cost‑effectiveness. Mtx, systemic steroids, CsA,
and acitretin were given alone or in combination.
Although retinoids are the drug of choice, [3,10,11]
Mtx was used in a majority of our patients due to
availability in the institute and lack of affordability.
Of 14 patients, 9 (6 acutely toxic and GPP of
pregnancy) were started on systemic steroids.
Prednisolone was given 40–60 mg/day and early
response was observed within a week and tapered
in 2–3 weeks except for 1 patient on 60 mg who
required 4 months for tapering.
261
[Downloaded free from http://www.jdrntruhs.org on Thursday, January 10, 2019, IP: 190.233.49.41]

Chintagunta, et al.: Pustular psoriasis

TABLE 1: DEMOGRAPHIC DATA AND RISK FACTORS

Age Sex Disease Clinical type Treatment given Risk factors Side Time Follow‑up
duration effects taken for
remission
22 M 2‑3 weeks De novo MtX 7.5 mg CRP elevated 4 months In remission
23 F 2‑3 weeks CPP 6 months MtX 7.5 mg Topical TEN, death ‑
steroids,
irritants
38 M 1‑2 months CPP Steroid + MtXacitretin 6 months In remission
28 F 3 weeks CPP 2 years Cyclosporine Pott’s spine 7 months In remission
100 mg bd
28 F Onset 28 De novo Prednisolone 30 Pregnancy Paronychia, 6 months Remission
weeks of mg, MtX 7.5 mg, granulation
pregnancy acitretin 25 mg tissue at
nail folds,
cheilitis
36 M 2‑3 weeks CPP 6 months Prednisolone 30 4‑6 months Relapse ‑ few
mg, MtX 7.5 mg psoriatic plaques
on the extremities
55 F 1‑2 weeks CPP 5 years Prednisolone 30 4 months Remission
mg + MtX 7.5 mg
40 M 2‑3 weeks CPP 10 years IV antibiotics CRP elevated 3 weeks Relapse ‑ few
plaques managing
with topicals
22 F 2 weeks CPP Prednisolone 30 mg Pregnancy, 4 weeks Lost for follow‑up
abnormal
liver enzymes
28 F 3 weeks CPP 3 years ART+acitretin 25 mg HIV 3 months Remission
38 M 2‑3 weeks CPP 8 years Prednisolone 30 Topical 4 months Plaque psoriasis
mg + MtX 10 mg steroids, ‑ extremities
salicylic acid
26 F Onset at De novo Prednisolone 60 mg, Pregnancy Paronychia, 4‑6 months Frequent relapses
28‑32 weeks cyclosporine (100 granulation ‑ involving
mg), acitretin (50 mg) tissue at limited areas
nail folds,
cheilitis
32 F 4‑6 months De novo MtX 7.5 mg Hypocalcemia 4 months Remission
9 M 4 months De novo MtX 7.5 mg CRP elevated 3 months Remission
MtX: Methotrexate; CRP: C‑reactive protein; CPP: Chronic plaque psoriasis; TEN: Toxic epidermal necrolysis

TABLE 2: TREATMENT DETAILS

Treatment modality No. of patients Associated/risk factors Side effects Outcome
MtX (7.5‑10 mg)+ 5 ‑ Mtx + 1 case ‑ TEN Remission in six patients.
corticosteroids prednisolone Two patients had
(prednisolone 40‑60 mg) 3 ‑ only Mtx frequent flares,
shifted to acitretin
Cyclosporine (3 mg/kg) 1 ‑ only CsA ATT for TB spine Nil Only CsA remission
1 ‑ CsA + Postpartum in 4 m
prednisolone CsA + CS ‑ freq flares,
shifted to acitretin
Corticosteroids 1 ‑ only CS Acutely ill patients 1 ‑ steroid Tapered in 3‑4 weeks
(prednisolone 30‑40 mg) 6 ‑ Mtx + CS Pregnancy dependency
1 ‑ CsA + CS
IV antibiotics 1 CRP elevated Nil Remission in 3 weeks
Acitretin (25 mg in one 4 HIV pts on HAART 2 ‑ paronychia, Remission in 4‑6 months
patient and 50 mg in 3 Granulation tissue
patients) at nail folds
MtX: Methotrexate; CsA: Cyclosporine; TEN: Toxic epidermal necrolysis; ATT: Anti tuberculous therapy; CRP: C‑reactive protein; HAART: Highly active antiretroviral
therapy

Among 14 patients, 8 patients were started on Early response with Mtx was observed in 2–4 weeks
Mtx 7.5–10 mg weekly along with folic acid (5 patients and complete remission in 4–6 months. Two patients
on Mtx and prednisolone combination and 3 only Mtx). had frequent flares and were shifted to acitretin.
262 Journal of Dr. NTR University of Health Sciences | Volume 7 | Issue 4 | October-December 2018
[Downloaded free from http://www.jdrntruhs.org on Thursday, January 10, 2019, IP: 190.233.49.41]
Chintagunta, et al.: Pustular psoriasis
Among patients on Mtx, one patient developed
erythema, generalized blistering, and painful skin
erosions after second dose of Mtx [Figure 5].
Nikolsky’s sign was positive, and biopsy was not
done as the patient refused to give consent. Based
on clinical findings, a diagnosis of toxic epidermal
necrolysis (TEN) was made. Mtx was stopped and
treated with systemic steroids and supportive therapy,
and the patient succumbed to death due to septicemia.
TEN has been reported with Mtx in patients with
psoriasis.[12]
CsA was given for two patients; one patient showed
complete remission. Another who had frequent flares
was shifted to acitretin. CsA showed early response
in less than 1 week and >90% response by 4 months.
Acitretin was given for four patients. In patients with
positive retroviral disease, acitretin was given along
with HAART therapy. Few case reports showed
successful treatment of HIV‑associated psoriasis with
acitretin.[13‑15] Two patients on Mtx and one on CsA
therapy with frequent flares were also shifted to
acitretin; all the three showed good response. Acitretin
showed early response by 2 weeks and 4–6 months
for eclearance. Acitretin was tapered gradually after
achieving 90% remission. Of four patients, one patient
frequently, that is, every 2, months came with three
to four small plaques with pustules and managed with
topical and one in complete remission. Two patients
became chronic plaque; there is no relapse of pustular
psoriasis and are on regular follow‑up.
In our study, steroids were followed by maintenance
therapy with Mtx/CsA/acitretin accordingly. Previous
studies with acitretin was found to be effective
in 84% when compared with treatment with Mtx
and CsA which were effective in 76% and 71% of
patients, respectively.[3] In our study, response with
Figure 5: Acute generalized pustular psoriasis, showing erythema,
erosions after second dose of methotrexate
Journal of Dr. NTR University of Health Sciences | Volume 7 | Issue 4 | October-December 2018
acitretin, Mtx, and CsA was 100%, 57.1%, and 50%,
respectively. Patients presented de novo and chronic
plaque psoriasis under regular follow‑up responded well
and those who were mismanaged by topical or oral
steroids took longer time to get remission. There was
no difference in the treatment. All the de novo cases
showed complete remission and there was no relapse
except one patient who had frequent flares of pustular
lesions involving the localized areas and managed with
topicals. GPP with a history of plaque psoriasis also
responded well and three patients have evidence of
localized small plaques manageable with topicals.
Limitations
Our study was limited to a small number of patients.
However, a larger prospective study is needed to draw
the conclusions.
CONCLUSION
GPP is not an uncommon disease with various
triggering factors. Our study also showed that acitretin
was found to be superior to CsA and Mtx in the
treatment of pustular psoriasis.
Declaration of patient consent
The authors certify that they have obtained all
appropriate patient consent forms. In the form the
patient(s) has/have given his/her/their consent for his/
her/their images and other clinical information to be
reported in the journal. The patients understand that
their names and initials will not be published and
due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
REFERENCES
1. Robinson A, Van Voorhees AS, Hsu S, Korman NJ, Lebwohl MG,
Bebo BF, et al. Treatment of pustular psoriasis: From the medical
board of the National Psoriasis Foundation. J Am Acad Dermatol
2012;67:279‑88.
2. Umezawa Y, Ozawa A, Kawasima T, Shimizu H, Terui T, Taqami H,
et al. Therapeutic guidelines for the treatment of generalized pustular
psoriasis (GPP) based on a proposed classification of disease severity.
Arch Dermatol Res 2003;295:43‑54.
3. Ozawa A, Ohkido M, Haruki Y, Kobayashi H, Ohkawara A, Ohno Y,
et al. Treatments of general pustular psoriasis: A multicenter study
in Japan. J Dermatol 1999;26:141‑9.
263
[Downloaded free from http://www.jdrntruhs.org on Thursday, January 10, 2019, IP: 190.233.49.41]

Chintagunta, et al.: Pustular psoriasis

4. Mengesha YM, Bennett ML. Pustular skin disorders: Diagnosis and
treatment. Am J Clin Dermatol 2002;3:389‑400.
5. Choon SE, Lai NM, Mohammad NA, Nanu NM, Tey KE, Chew SF.
Clinical profile, morbidity, and outcome of adult‑onset generalized
pustular psoriasis: Analysis of 102 cases seen in a tertiary hospital in
Johor, Malaysia. Int J Dermatol 2014;53:676‑84.
6. Tay YK, Tham SN. The profile and outcome of pustular psoriasis in
Singapore: A report of 28 cases. Int J Dermatol 1997;36:266‑71.
7. Brightman L, Stefanato CM, Bhawan J, Phillips TJ. Third trimester
impetigo herpetiformis treated with cyclosporine. JAM Acad Dermatol
2007;56:S62‑4.
8. Hazarika D. Generalized pustular psoriasis of pregnancy successfully
treated with cyclosporine. Indian J Dermatol Venereol Leprol 2009;75:638.
9. Rather S, Arif T, Hassan I. Impetigo herpetiformis: A challenging
scenario to the treating dermatologist: A case series. Egypt Dermatol
Online J 2014;10:5.
10. Augey F, Renaudier P, Nicolas JF. Generalized pustular
psoriasis (Zumbusch): A French epidemiological survey. Eur J
Dermatol 2006;16:669‑73.
11. Magis NL, Blummel JJ, Kerhof PC, Gorritsen RM. The treatment of
psoriasis with etretinate and acitretin: A follow up of actual use. Eur
J Dermatol 2000;10:517‑21.
12. Rogers SC, McKee PH. Toxic epidermal necrolysis in two patients
with pustular psoriasis. Br J Dermatol 1977;96:323‑6.
13. Jeong YS, Kim MS, Shin JS, Cho JK, Lee H, Kim HJ, et al. A case of
severe HIV‑associated psoriasis successfully treated with acitretin
therapy. Infect Chemother 2014;46:115‑9.
14. Menon K, Van Voorhees AS, Bebo BF Jr, Gladman DD, Hsu S,
Kalb RE, et al. National Psoriasis Foundation. Psoriasis in patients
with HIV infection: From the medical board of the National Psoriasis
Foundation. J Am Acad Dermatol 2010;62:291‑9.
15. Buccheri L, Katchen BR, Karter AJ, Cohen SR. Acitretin therapy is
effective for psoriasis associated with human immunodeficiency virus
infection. Arch Dermatol 1997;133:711-5.

264 Journal of Dr. NTR University of Health Sciences | Volume 7 | Issue 4 | October-December 2018