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  1. C100 医学部/医学系研究科
  2. C100a 雑誌掲載論文
  3. 学術雑誌

Generalized Pustular Psoriasis: Clinical Management and Update on Autoinflammatory Aspects

http://hdl.handle.net/2237/00032517
http://hdl.handle.net/2237/00032517
abbd28aa-5daa-4295-9aee-5c0827e8b550
名前 / ファイル ライセンス アクション
R2_main R2_main text_191119_clean (223.5 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2020-07-15
タイトル
タイトル Generalized Pustular Psoriasis: Clinical Management and Update on Autoinflammatory Aspects
言語 en
著者 Takeichi, Takuya

× Takeichi, Takuya

WEKO 100618

en Takeichi, Takuya

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Akiyama, Masashi

× Akiyama, Masashi

WEKO 100619

en Akiyama, Masashi

Search repository
アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
権利
言語 en
権利情報 “This is a post-peer-review, pre-copyedit version of an article published in [American Journal of Clinical Dermatology]. The final authenticated version is available online at: http://dx.doi.org/10.1007/s40257-019-00492-0”.
抄録
内容記述 Generalized pustular psoriasis (GPP) is a chronic, systemic inflammatory disease accompanied by high fever and general malaise. Diffuse erythema and swelling of the extremities occur, with multiple sterile pustules all over the body in GPP patients. GPP often relapses over the lifetime and can be life-threatening. Recent discoveries of the underlying molecular genetic basis of many cases of this disorder have provided major advances to clinicians and researchers towards an understanding of the pathomechanism of GPP. However, the therapeutic management of GPP still faces many challenges and much uncertainty, and an evidence-based review summarizing the available clinical data on the management of this heterogeneous disease is needed. The present review addresses challenges regarding the precise clinical diagnosis and evaluation of clinical symptoms in GPP. In addition, we update and briefly summarize the current understanding of molecular pathomechanisms behind GPP as an autoinflammatory keratinization disease. Recent publications have clarified the genetic backgrounds of patients with GPP and ethnic differences in predisposing factors. Although there are ethnic differences in the prevalences of these pathogenic alleles, from recent reports, at most 60.5% (IL36RN), 5.9% (CARD14), and 10.8% (AP1S3) of GPP patients have the mutations/variations of these genes. All the reported biologics studied seemed effective and relatively well tolerated. Although it is difficult to evaluate therapeutic efficacy from studies on just a few cases, recent findings suggest that biologics can be a useful, powerful tool for controlling skin and systemic inflammation in GPP and for improving the quality of life of GPP patients.
言語 en
内容記述タイプ Abstract
内容記述
内容記述 ファイル公開:2021/04/01
言語 ja
内容記述タイプ Other
出版者
言語 en
出版者 Springer
言語
言語 eng
資源タイプ
資源タイプresource http://purl.org/coar/resource_type/c_6501
タイプ journal article
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
DOI
関連タイプ isVersionOf
識別子タイプ DOI
関連識別子 https://doi.org/10.1007/s40257-019-00492-0
ISSN(print)
収録物識別子タイプ PISSN
収録物識別子 1175-0561
書誌情報 en : American Journal of Clinical Dermatology

巻 21, 号 2, p. 227-236, 発行日 2020-04
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