442 European consensus statement on phenotypes of pustular psoriasis

Navarini, Alexander; Burden, David; Capon, Francesca; Mrowietz, Ulrich; Puig, L.; Smith, Cara Lea; Barker, Jane

doi:10.1016/j.jid.2016.06.462

Cited by 2 publications

(4 citation statements)

References 28 publications

(34 reference statements)

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“…To our knowledge, this is the first systematic study of the factors associated with PPP severity. This study builds on previous work from our network, which enabled the definition of consensus diagnostic criteria for PPP 7 and suggested that the disease is genetically different from other forms of pustular psoriasis. 2 Our investigation noted key epidemiologic features of PPP, such as the late age of onset and sex bias (male-female ratios were >1:3.5 in both cohorts).…”

Section: Discussionmentioning

confidence: 71%

“…Pustular psoriasis was always diagnosed by a dermatologist, based on clinical examination and/or the ERASPEN consensus criteria. 7 The observation of sterile, macroscopically visible pustules on palms or soles was the main inclusion criterion. Conversely, the presence of pustules restricted to the edges of psoriatic plaques represented an exclusion criterion.…”

Section: Patientsmentioning

confidence: 99%

“…The lesions, which can occur on a background of normal or inflamed skin, are persistent (>3 months), painful, and disabling, and can be accompanied by fissures, pruritus, and a burning sensation. 7 Comorbidities are also common, as affected individuals are at increased risk of psoriasis vulgaris, psoriatic arthritis, and autoimmune thyroid disease. 8 While PPP can profoundly impact quality of life, the factors underlying variable disease severity have not been investigated.…”

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confidence: 99%

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Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis

et al. 2020

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; for the ERASPEN consortium and the APRICOT and PLUM study team IMPORTANCE Although palmoplantar pustulosis (PPP) can significantly impact quality of life, the factors underlying disease severity have not been studied. OBJECTIVE To examine the factors associated with PPP severity. DESIGN, SETTING, AND PARTICIPANTS An observational, cross-sectional study of 2 cohorts was conducted. A UK data set including 203 patients was obtained through the Anakinra in Pustular Psoriasis, Response in a Controlled Trial (2016-2019) and its sister research study Pustular Psoriasis, Elucidating Underlying Mechanisms (2016-2020). A Northern European cohort including 193 patients was independently ascertained by the European Rare and Severe Psoriasis Expert Network (2014-2017). Patients had been recruited in secondary or tertiary dermatology referral centers. All patients were of European descent. The PPP diagnosis was established by dermatologists, based on clinical examination and/or published consensus criteria. The present study was conducted from October 1, 2014, to March 15, 2020. MAIN OUTCOMES AND MEASURES Demographic characteristics, comorbidities, smoking status, Palmoplantar Pustulosis Psoriasis Area Severity Index (PPPASI), measuring severity from 0 (no sign of disease) to 72 (very severe disease), or Physician Global Assessment (PGA), measuring severity as 0 (clear), 1 (almost clear), 2 (mild), 3 (moderate), and 4 (severe). RESULTS Among the 203 UK patients (43 men [21%], 160 women [79%]; median age at onset, 48 [interquartile range (IQR), 38-59] years), the PPPASI was inversely correlated with age of onset (r = −0.18, P = .01). Similarly, in the 159 Northern European patients who were eligible for inclusion in this analysis (25 men [16%], 134 women [84%]; median age at onset, 45 [IQR, 34-53.3] years), the median age at onset was lower in individuals with a moderate to severe PGA score (41 years [IQR, 30.5-52 years]) compared with those with a clear to mild PGA score (46.5 years [IQR, 35-55 years]) (P = .04). In the UK sample, the median PPPASI score was higher in women (9.6 [IQR, 3.0-16.2]) vs men (4.0 [IQR, 1.0-11.7]) (P = .01). Likewise, moderate to severe PPP was more prevalent among Northern European women (57 of 134 [43%]) compared with men (5 of 25 [20%]) (P = .03). In the UK cohort, the median PPPASI score was increased in current smokers (10.7 [IQR, 4.2-17.5]) compared with former smokers (7 [IQR, 2.0-14.4]) and nonsmokers (2.2 [IQR, 1-6]) (P = .003). Comparable differences were observed in the Northern European data set, as the prevalence of moderate to severe PPP was higher in former and current smokers (51 of 130 [39%]) compared with nonsmokers (6 of 24 [25%]) (P = .14). CONCLUSIONS AND RELEVANCE The findings of this study suggest that PPP severity is associated with early-onset disease, female sex, and smoking status. Thus, smoking cessation intervention might be beneficial.

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Section: Discussionmentioning

confidence: 71%

Section: Patientsmentioning

confidence: 99%

mentioning

confidence: 99%

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Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis

et al. 2020

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“…9,11,13 In contrast, the European consensus on pustular psoriasis defines GPP as the presence of macroscopically visible sterile pustules on nonacral skin, with or without systemic inflammation and with or without associated psoriasis. 14 By this definition, neither systemic manifestation nor histologic confirmation is necessary for a diagnosis of GPP. Hence, establishing an internationally accepted standardized definition of GPP based on agreed-upon diagnostic criteria is essential for meaningful research.…”

mentioning

confidence: 99%

International Consensus Definition and Diagnostic Criteria for Generalized Pustular Psoriasis From the International Psoriasis Council

Choon,

van de Kerkhof,

Gudjonsson

et al. 2024

JAMA Dermatol

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ImportanceGeneralized pustular psoriasis (GPP) lacks internationally accepted definitions and diagnostic criteria, impeding timely diagnosis and treatment and hindering cross-regional clinical and epidemiological study comparisons.ObjectiveTo develop an international consensus definition and diagnostic criteria for GPP using the modified Delphi method.Evidence ReviewThe rarity of GPP presents a challenge in acquiring comprehensive published clinical data necessary for developing standardized definition and criteria. Instead of relying on a literature search, 43 statements that comprehensively addressed the fundamental aspects of the definitions and diagnostic criteria for GPP were formulated based on expert reviews of 64 challenging GPP cases. These statements were presented to a panel of 33 global GPP experts for voting, discussion, and refinements in 2 virtual consensus meetings. Consensus during voting was defined as at least 80% agreement; the definition and diagnostic criteria were accepted by all panelists after voting and in-depth discussion.FindingsIn the first and second modified Delphi round, 30 (91%) and 25 (76%) experts participated. In the initial Delphi round, consensus was achieved for 53% of the statements, leading to the approval of 23 statements that were utilized to develop the proposed definitions and diagnostic criteria for GPP. During the second Delphi round, the final definition established was, “Generalized Pustular Psoriasis is a systemic inflammatory disease characterized by cutaneous erythema and macroscopically visible sterile pustules.” It can occur with or without systemic symptoms, other psoriasis types, and laboratory abnormalities. GPP may manifest as an acute form with widespread pustules or a subacute variant with an annular phenotype. The identified essential criterion was, “Macroscopically visible sterile pustules on erythematous base and not restricted to the acral region or within psoriatic plaques.”Conclusions and RelevanceThe achievement of international consensus on the definition and diagnostic criteria for GPP underscores the importance of collaboration, innovative methodology, and expert engagement to address rare diseases. Although further validation is needed, these criteria can serve as a reference point for clinicians, researchers, and patients, which may contribute to more accurate diagnosis and improved management of GPP.

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442 European consensus statement on phenotypes of pustular psoriasis

Cited by 2 publications

References 28 publications

Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis

Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis

International Consensus Definition and Diagnostic Criteria for Generalized Pustular Psoriasis From the International Psoriasis Council

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