Clinical and genetic differences between pustular psoriasis subtypes

Twelves, Sophie; Mostafa, Alshimaa; Dand, Nick; Burri, Elias; Farkas, Katalin; Wilson, Rebecca; Cooper, Hywel L; Irvine, Alan D.; Oon, Hazel H; Kingo, Külli; Kõks, Sulev; Mrowietz, Ulrich; Puig, Luís; Reynolds, Nick; Tan, Eugene; Tanew, Adrian; Torz, Kaspar; Trattner, Hannes; Valentine, Mark C.; Wahie, Shyamal; Warren, Richard B; Wright, Andrew; Bata-Csorgo, Zsuzsanna; Széll, Márta; Griffiths, Christopher E.M.; Burden, A. D.; Choon, Siew Eng; Smith, Catherine; Barker, Jonathan; Navarini, Alexander A.; Capon, Francesca

doi:10.1016/j.jaci.2018.06.038

Cited by 192 publications

(344 citation statements)

References 21 publications

(37 reference statements)

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“…Mouse model results also support this view: Il36rn-null mice never show the disease spontaneously, but need imiquimod as a potential trigger to show GPP-like phenotypes (10). IL36RN mutations are occasionally found in GPP patients with psoriasis, although GPP patients without psoriasis seem to have IL36RN mutations more frequently (4,9). In addition, Arakawa et al (11) reported that induced IL36RN levels are lower in both GPP patients with and without IL36RN mutations, and proposed "IL36RN insufficiency" in GPP.…”

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confidence: 70%

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Autoinflammatory Keratinization Diseases (AiKDs): Expansion of Disorders to Be Included

Akiyama

2020

Front. Immunol.

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confidence: 70%

“…It remains controversial as to whether GPP should be considered an AiKD. Many patients with GPP, acrodermatitis continua, or impetigo herpetiformis have been reported to have IL36RN mutations (3)(4)(5)(6)(7)(8)(9). GPP shows massive neutrophil infiltration leading to pustulosis and occasionally erosive skin.…”

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confidence: 99%

Autoinflammatory Keratinization Diseases (AiKDs): Expansion of Disorders to Be Included

Akiyama

2020

Front. Immunol.

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“…The large collections of intraepidermal and subcorneal neutrophils is the main characteristic of pustular psoriasis skin lesions (43). It is recognized now that adaptive immune responses predominate in chronic plaque psoriasis, whereas innate and autoinflammatory responses dominate in GPP (44).…”

Section: Discussionmentioning

confidence: 99%

Neutrophil exosomes enhance the skin autoinflammation in generalized pustular psoriasis via activating keratinocytes

et al. 2019

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Generalized pustular psoriasis (GPP) is a rare and severe inflammatory skin disease that can be life‐threatening. Gene mutations are found in some cases, but its immune pathogenesis is largely unknown. Here, we observed that the neutrophil:lymphocyte ratio in patients with GPP was higher than that in healthy controls and decreased after effective treatment. Neutrophils isolated from patients with GPP induced higher expressions of inflammatory genes including IL‐1β, IL‐36G, IL‐18, TNF‐α, and C‐X‐C motif chemokine ligands in keratinocytes than normal neutrophils did. Moreover, neutrophils from patients with GPP secreted more exosomes than controls, which were then rapidly internalized by keratinocytes, increasing the expression of these inflammatory molecules via activating NF‐κB and MAPK signaling pathways. The proteomic profiles in neutrophil exosomes further characterized functional proteins and identified olfactomedin 4 as the critical differentially expressed protein that mediates the autoimmune inflammatory responses of GPP. These results demonstrate that neutrophil exosomes have an immune‐regulatory effect on keratinocytes, which modulates immune cell migration and autoinflammation in GPP.—Shao, S., Fang, H., Zhang, J., Jiang, M., Xue, K., Ma, J., Zhang, J., Lei, J., Zhang, Y., Li, B., Yuan, X., Dang, E., Wang, G. Neutrophil exosomes enhance the skin autoinflammation in generalized pustular psoriasis via activating keratinocytes. FASEB J. 33, 6813–6828 (2019). http://www.fasebj.org

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“…Further, while mutations in the IL36-RN, AP1S3, and CARD14 genes have been reported in GPP, with some ethnic differences [7], IL36RN does not appear to play a significant role in either Asian or European PPP patients [8][9][10][11]. Mutations in the CARD14 gene have been described in PPP patients in Europe [8].…”

Section: Introductionmentioning

confidence: 94%

The clinical, humanistic, and economic burden of palmoplantar pustulosis: a structured review

Kharawala¹,

Golembesky

Bohn³

et al. 2020

Expert Review of Clinical Immunology

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Introduction: Palmoplantar pustulosis (PPP) is a chronic, relapsing and refractory disease characterized by sterile pustules appearing on the palms and/or soles, accompanied by erythema, blistering, scales and/or keratinization. The overall burden of PPP in terms of its clinical impact, effect on patients and families, and economic consequences has not previously been investigated in a structured manner. Areas covered: A structured search focused on identification of studies in PPP using specific search terms in PubMed and EMBASE® from 2005 onwards, with additional back-referencing and pragmatic searches. Outcomes of interest included clinical burden, humanistic burden, and economic burden. Expert opinion: In cross-sectional studies, approximately 75% of all PPP patients suffer from active disease, with risk of relapse remaining constant over time. Patients' health-related quality of life is significantly impaired, as expected for a disease affecting hands and feet. Tools have been described that assess the clinical as well as patient-reported burden of PPP; their performance in larger cohorts and/or clinical trials remains to be investigated. The key data limitations identified include inconsistent definitions for characterizing remission/relapse, and limited humanistic and economic burden data; future studies are required to address these evidence gaps.ARTICLE HISTORY (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited, and is not altered, transformed, or built upon in any way. disease over time, including risk factors, clinical outcomes, and comorbidities); (2) Humanistic burden (functioning, quality of life [QoL], psychosocial burden, adherence/preference/satisfaction with treatment and caregiver burden); and (3) Economic burden (drivers of resource use and cost, actual costs and utilities). In the sister publication published in the same edition [2], the burden of disease of GPP is characterized in a similar way.

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Clinical and genetic differences between pustular psoriasis subtypes

Abstract: The analysis of an unparalleled resource revealed key clinical and genetic differences between patients with PPP and those with GPP.

Cited by 192 publications

References 21 publications

Autoinflammatory Keratinization Diseases (AiKDs): Expansion of Disorders to Be Included

Autoinflammatory Keratinization Diseases (AiKDs): Expansion of Disorders to Be Included

Neutrophil exosomes enhance the skin autoinflammation in generalized pustular psoriasis via activating keratinocytes

The clinical, humanistic, and economic burden of palmoplantar pustulosis: a structured review

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