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Pustular Psoriasis: From Pathophysiology to Treatment.

ABSTRACT: Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (IL)-17/IL-23 axis, PP seems to be characterized by an intense inflammatory response resulting from innate immunity hyperactivation, with prominent involvement of the IL-36 axis. Some nosological aspects of PP are still controversial and debated. Moreover, owing to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited. Recent progresses in the identification of genetic mutations and immunological mechanisms have promoted a better understanding of PP pathogenesis and might have important consequences on diagnostic refinement and treatment. In this narrative review, current findings in the pathogenesis, classification, clinical features, and therapeutic management of PP are briefly discussed.

SUBMITTER: Genovese G

PROVIDER: S-EPMC8698272 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature

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Pustular Psoriasis: From Pathophysiology to Treatment.

Genovese Giovanni G Moltrasio Chiara C Cassano Nicoletta N Maronese Carlo Alberto CA Vena Gino Antonio GA Marzano Angelo Valerio AV

Biomedicines 20211123 12

Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (I ...[more]

PMID: 34944562

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Project description:Pustular psoriasis is an unusual form of psoriasis that frequently presents clinical challenges for dermatologists. The condition presents with pustules on an erythematous background and has two distinct subtypes: localized disease on the palms and soles, called palmoplantar pustulosis (PPP), and generalized pustular psoriasis (GPP). The involvement of the fingers, toes, and nails is defined as a separate localized variant, acrodermatitis continua of Hallopeau, and is now thought to be a subset of PPP. The rarity of pustular psoriasis frequently makes the correct diagnosis problematic. In addition, treatment is limited by a relative lack of evidence-based therapeutic options. Current management is often based on existing therapies for standard plaque psoriasis. However, there remains a need for treatments with high, sustained efficacy and a rapid onset of action in pustular psoriasis. Recent advances in understanding of the pathogenesis of pustular psoriasis have provided insights into potential therapies. Treatment of pustular psoriasis is generally determined by the extent and severity of disease, and recent years have seen an increasing use of newer agents, including biologic therapies. Current classes of biologic therapies with US Food and Drug Administration and European Medicines Agency approval for treatment of moderate-to-severe plaque psoriasis in the USA (and elsewhere) include tumor necrosis factor alpha inhibitors (adalimumab, certolizumab pegol, etanercept, infliximab), interleukin (IL)-17 inhibitors (brodalumab, ixekizumab, secukinumab), an IL-12/23 inhibitor (ustekinumab), and IL-23 inhibitors (guselkumab, risankizumab, tildrakizumab). Recently, specific inhibitors of the IL-36 pathway have been evaluated in GPP and PPP, including spesolimab, an IL-36 receptor inhibitor which has shown promising results in GPP. The emerging drugs for pustular psoriasis offer the possibility of rapid and effective treatment with lower toxicities than existing therapies. Further research into agents acting on the IL-36 pathway and other targeted therapies has the potential to transform the future treatment of patients with pustular psoriasis. This article reviews the clinical features of PPP and GPP, and current understanding of the genetics and immunopathology of these conditions; it also provides an update on emerging treatments.

Project description:ImportancePalmoplantar pustulosis (PPP) and palmoplantar plaque psoriasis with pustules remain challenging to treat. Studies suggest that an interleukin 17 or interleukin 36 loop acts synergistically in these diseases to induce palmoplantar pustules.ObjectiveTo assess the effectiveness of bimekizumab in treating PPP and palmoplantar plaque psoriasis with pustules.Design, setting, and participantsThis case series involved 21 adults with PPP (11 patients) or palmoplantar plaque psoriasis with pustules (10 patients) treated at 1 of 7 tertiary dermatological centers in France from September 2022 through June 2023. All patients treated with bimekizumab for at least 3 months were included in the analyses.Main outcomes and measuresThe main outcome was the posttreatment Investigator Global Assessment (IGA), scored as 0 (complete clearance), 1 (almost clear), 2 (mild), 3 (moderate), or 4 (severe). When relevant, evolution of joint pain and nail involvement was reported. Tolerance and potential adverse events were noted.ResultsA total of 21 patients (mean [range] age, 46 [24-68] years; 19 females) were included. Eleven patients had isolated PPP, and 10 had palmoplantar plaque psoriasis with pustules. All of them, except 2 who received bimekizumab as first systemic therapy, had not responded to at least 1 systemic treatment (median [range], 3 [1-7] treatments), and/or had adverse events leading to the discontinuation of the treatment. Complete clearance (IGA score, 0) was achieved by 17 patients in 1 to 4 months. Three patients achieved an IGA score of 1, and 1 achieved an IGA score of 2. Three patients with PPP also presented with acrodermatitis continua of Hallopeau. Nail involvement showed 50% to 70% improvement after 4 to 6 months of bimekizumab treatment for these 3 patients. Two patients had SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome; both had complete clearance of skin lesions associated with joint pain improvement. Four patients (19%) with candidiasis were successfully treated with oral antifungal agents. None of the patients had to stop bimekizumab treatment due to adverse events.Conclusions and relevanceThe findings of this case series suggest that bimekizumab could be an appealing approach for treating PPP, palmoplantar plaque psoriasis with pustules, and SAPHO syndrome. Prospective randomized placebo-controlled clinical trials are needed to confirm these encouraging initial results.

Project description:BackgroundGeneralized Pustular Psoriasis (GPP) is an autoinflammatory, multisystemic disease, characterized by widespread eruption of neutrophilic pustules on erythematous base, accompanied by systemic symptoms such as fever, leukocytosis, arthralgia, and general malaise. Globally, the disease is rare, particularly in children. If not adequately diagnosed and treated, systemic inflammation and multiorgan involvement can be life-threatening. The pathogenesis of GPP mainly involves the innate immune system, with inflammatory processes and neutrophil activation driven primarily by IL-36, but also by IL-1, TNF-alpha, IL-17 A. In particular, IL-17 A acts as a potent inducer of neutrophil recruitment. We report the case of a 7-years-old girl with GPP successfully treated with Ixekizumab, an IL-17 A antagonist.Case presentationA 7-years-old girl with an history of plaque psoriasis came to our attention for the sudden appearance of erythematous patches surmounted by pustules on the trunk and lower limbs, following repeated episodes of purulent tonsillitis. We started therapy with cyclosporine at a dosage of 3,5 mg/kg/day, with no clinical benefit and progression of manifestations to a sub-erythrodermic state after 2 weeks. Blood tests showed neutrophilic leukocytosis, and the patient experienced hyperpyrexia and malaise. Since ixekizumab was recently approved for pediatric use in patients with moderate to severe plaque psoriasis, we started therapy with 80 mg Ixekizumab, combined with prednisone at a dosage of 12.5 mg/day, gradually tapered until discontinuation after 15 days. A second dose of Ixekizumab 40 mg was administered at week-4, according to the indication of ixekizumab in pediatric plaque psoriasis. At week-8 the patient achieved complete remission of skin manifestations and normalization of blood count. After achieving a stable remission, at week 36 we decided to increase the administration interval to 6 weeks. The patient is still on therapy with ixekizumab 40 mg every 6 weeks, maintaining complete remission during a 52-week follow-up, without safety concerns.ConclusionsThis report supports the use of ixekizumab as a safe and effective option, both in the short and long-term, in the treatment of GPP, even at pediatric age. Larger studies are needed to confirm this positive, real-life experience.

Dataset Information

Pustular Psoriasis: From Pathophysiology to Treatment.

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Pustular Psoriasis: From Pathophysiology to Treatment.

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