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ORIGINAL ARTICLE
Year : 2018  |  Volume : 7  |  Issue : 4  |  Page : 259-264

Pustular psoriasis – Clinical study in a tertiary care center


1 Department of DVL, Government Medical College, Nizamabad, India
2 Deparment of DVL, Gandhi Medical College and Hospital, Hyderabad, Telangana, India

Date of Web Publication10-Jan-2019

Correspondence Address:
Dr. Sudha Rani Chintagunta
Plot No. 5, Jupiter Colony, Kakaguda, Kharkhana, Secunderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_109_17

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  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.

Keywords: Clinical profile, pustular psoriasis, therapeutic response


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

How to cite this URL:
Chintagunta SR, Damarla SV, Gopidi PJ, Arakkal G. Pustular psoriasis – Clinical study in a tertiary care center. J NTR Univ Health Sci [serial online] 2018 [cited 2019 Nov 13];7:259-64. Available from: http://www.jdrntruhs.org/text.asp?2018/7/4/259/249821




  Introduction Top


Pustular psoriasis is an uncommon form of psoriasis consisting of sheets of pustules on erythematous background. It can occur de novo or progress from other forms of psoriasis. The course of generalized pustular psoriasis (GPP) varies from acute, subacute, or chronic. The acute type (von Zumbusch variant) is associated with constitutional signs and symptoms and systemic complications. The common subacute and chronic types present with skin symptoms only. There are limited data in literature about guidelines for the therapy of pustular psoriasis. The treatment 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],[3],[4] Mtx was used in a majority of our patients due to nonavailability of retinoids in the institute.


  Materials and Methods Top


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 Top


Of 14 patients, 9 were females and 5 were males. 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.
Figure 1: Acute generalized pustular psoriasis with geographic tongue

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Table 1: Demographic Data and Risk Factors

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Figure 2: Generalized pustular psoriasis of pregnancy, response to systemic steroids

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Figure 3: Nail fold inflammation and ulceration following acitretin

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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].
Table 2: Treatment Details

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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 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].
Figure 4: GPP response to systemic steroids and methotrexate

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  Discussion Top


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.

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],[8],[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.

Among 14 patients, 8 patients were started on Mtx 7.5–10 mg weekly along with folic acid (5 patients on Mtx and prednisolone combination and 3 only Mtx). Early response with Mtx was observed in 2–4 weeks and complete remission in 4–6 months. Two patients had frequent flares and were shifted to acitretin.

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]
Figure 5: Acute generalized pustular psoriasis, showing erythema, erosions after second dose of methotrexate

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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],[14],[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 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 Top


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 Top

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.  Back to cited text no. 1
    
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.  Back to cited text no. 2
    
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.  Back to cited text no. 3
    
4.
Mengesha YM, Bennett ML. Pustular skin disorders: Diagnosis and treatment. Am J Clin Dermatol 2002;3:389-400.  Back to cited text no. 4
    
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.  Back to cited text no. 5
    
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.  Back to cited text no. 6
    
7.
Brightman L, Stefanato CM, Bhawan J, Phillips TJ. Third trimester impetigo herpetiformis treated with cyclosporine. JAM Acad Dermatol 2007;56:S62-4.  Back to cited text no. 7
    
8.
Hazarika D. Generalized pustular psoriasis of pregnancy successfully treated with cyclosporine. Indian J Dermatol Venereol Leprol 2009;75:638.  Back to cited text no. 8
[PUBMED]  [Full text]  
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.  Back to cited text no. 9
    
10.
Augey F, Renaudier P, Nicolas JF. Generalized pustular psoriasis (Zumbusch): A French epidemiological survey. Eur J Dermatol 2006;16:669-73.  Back to cited text no. 10
    
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.  Back to cited text no. 11
    
12.
Rogers SC, McKee PH. Toxic epidermal necrolysis in two patients with pustular psoriasis. Br J Dermatol 1977;96:323-6.  Back to cited text no. 12
    
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.  Back to cited text no. 13
    
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.  Back to cited text no. 14
    
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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.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2]



 

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