Biologic Therapy in Erythrodermic and Pustular Psoriasis

March 2014 | Volume 13 | Issue 3 | Original Article | 342 | Copyright © March 2014


Ethan C. Levin MD,a Maya Debbaneh BA,b John Koo MD,a and Wilson Liao MDa

aDepartment of Dermatology, Psoriasis and Skin Treatment Center, University of California, San Francisco, San Francisco, CA bUniversity of Irvine, School of Medicine, Irvine, CA

Abstract
BACKGROUND: The efficacy of biologic therapy in treating plaque-type psoriasis is well documented. However, there is less data for use in other psoriasis subtypes, such as erythrodermic and generalized pustular psoriasis.
OBJECTIVE: We sought to review the safety and efficacy of biologic medications in the treatment of these severe subtypes of psoriasis and to identify strategies to help clinicians optimally manage these patients.
METHODS: We searched Pubmed for English language literature that assessed the use of biologic medication to treat erythrodermic or generalized pustular psoriasis.
RESULTS: The primary literature included cases reports, cases series, and open-label, uncontrolled trials. There were no head-to-head studies or other controlled trials. In both erythrodermic and generalized pustular psoriasis, infliximab was used to treat over half of the reported cases. Other biologic medications that were successfully used included etanercept, ustekinumab, adalimumab, and anakinra. Most cases reported improvement with biologic therapy. Serious adverse events were reported in 10-12% of the patients.
CONCLUSION: Although the evidence is limited, biologic therapy appears to be effective in treating erythrodermic and generalized pustular psoriasis. In order to assess the comparative efficacy and safety of the biologic medications, larger controlled studies are needed.

J Drugs Dermatol. 2014;13(3):342-354.

INTRODUCTION

Biologic medications have been thoroughly tested in clinical trials and have well documented efficacy in the treatment of chronic plaque psoriasis. However, there is less data for use in other psoriasis subtypes, such as erythrodermic and generalized pustular psoriasis. Traditional systemic therapy for erythrodermic and generalized pustular psoriasis includes cyclosporine, acitretin, and methotrexate. The evidence for the use of these agents is based largely on retrospective reviews, case series, and uncontrolled studies.1-9
Although often effective, these traditional medications have limitations. Acitretin and methotrexate are contraindicated in patients with severely impaired hepatic function and in pregnancy. Cyclosporine should be avoided in patients with kidney disease and in those with uncontrolled hypertension. In contrast, biologic medications do not have any absolute contraindications in the aforementioned patients. Further, these medications represent an opportunity to enhance therapeutic efficacy in the most difficult-to-treat cases of psoriatic disease. While there are consensus guidelines published by the National Psoriasis Foundation for the treatment of erythrodermic and generalized pustular psoriasis, they do not include information on all the biologic medications, especially those that were most recently approved.10, 11 Further, there has never been a review that focuses specifically on the merits of biologic agents for the treatment of these rare but potentially life-threatening subtypes of psoriasis.
Our aim was to review the safety and efficacy of biologic medications in the treatment of erythrodermic and generalized pustular psoriasis. We hope to make practical recommendations for clinicians to optimally manage patients with the most severe phenotypes of psoriasis in the fast-evolving landscape of psoriasis therapy.

METHODS

We searched Pubmed for English language literature that assessed the use of biologic medication to treat erythrodermic or generalized pustular psoriasis. The search terms included “[drug name]” AND “erythrodermic psoriasis” OR “generalized pustular psoriasis”. The following drug names were used: ustekinumab, adalimumab, etanercept, infliximab, and anakinra. Only literature that presented primary data was considered. A citation review of relevant articles was also performed.

Cases Identified

A total of 45 publications were identified that reported the use of a biologic medication to treat erythrodermic or generalized pustular psoriasis. The majority of reports were single cases or case series. There were three open-label, uncontrolled, clinical trials and one retrospective, multicenter, chart review.12-15 In total, biologic therapy was used in 91 cases of erythrodermic psoriasis and 55 cases of generalized pustular psoriasis (Table 1). Patients with erythrodermic psoriasis were an average of 37 years old, 28% were female, 23% had used prior biologic therapy and 14% had used multiple prior biologic therapies. Those with generalized pustular psoriasis were an average of 44 years old, 73% were female, 40% had used prior biologic therapy and 17% had used multiple prior biologic therapies. On average, patients in both groups failed at least two traditional systemic medications (cyclosporine, methotrexate, or acitretin). The biologic medications used to treat both subtypes of psoriasis included infliximab, adalimumab, etanercept, and ustekinumab. In addition, anakinra was used to treat two cases of generalized pustular psoriasis.16

Efficacy in Erythrodermic Psoriasis

With respect to erythrodermic psoriasis, infliximab was used in over half of the reported cases (57%) (Table 1). In order of frequen-
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