Generalized pustular psoriasis: overview of the status quo and results of a panel discussion

被引：20

作者：

Reich, Kristian ^{[1
]}

Augustin, Matthias ^{[1
]}

Gerdes, Sascha ^{[2
]}

Ghoreschi, Kamran ^{[3
,4
,5
]}

Kokolakis, Georgios ^{[3
,4
,5
]}

Moessner, Rotraut ^{[6
]}

Mrowietz, Ulrich ^{[2
]}

Navarini, Alexander A. ^{[7
]}

Pinter, Andreas ^{[8
]}

Schaekel, Knut ^{[9
]}

Staubach, Petra ^{[10
]}

Sticherling, Michael ^{[11
]}

Thaci, Diamant ^{[12
]}

Wilsmann-Theis, Dagmar ^{[13
]}

机构：

[1] Univ Med Ctr Hamburg Eppendorf UKE, Inst Hlth Serv Res Dermatol & Nursing, Hamburg, Germany

[2] Univ Med Ctr Schleswig Holstein, Clin Dermatol Venereol & Allergol, Psoriasis Ctr, Campus Kiel, Kiel, Germany

[3] Charite Univ Med Berlin, Dept Dermatol Venereol & Allergol, Berlin, Germany

[4] Free Univ Berlin, Berlin, Germany

[5] Humboldt Univ, Berlin, Germany

[6] Univ Med Ctr Gottingen, Dept Dermatol Venerol & Allergol, Gottingen, Germany

[7] Univ Basel Hosp, Dept Dermatol, Basel, Switzerland

[8] Goethe Univ, Dept Dermatol Venereol & Allergol, Frankfurt, Germany

[9] Univ Heidelberg Hosp, Dept Dermatol, Heidelberg, Germany

[10] Univ Med Ctr Mainz, Dept Dermatol, Mainz, Germany

[11] Friedrich Alexander Univ, Dept Dermatol, Deutsch Zentrum Immuntherapie, Erlangen, Germany

[12] Univ Hosp Schleswig Holstein, Inst & Comprehens Ctr Inflammat Med, Campus Lubeck, Lubeck, Germany

[13] Univ Hosp Bonn, Dept Dermatol & Allergy, Bonn, Germany

来源：

JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT | 2022年 / 20卷 / 06期

关键词：

JAPANESE PATIENTS; MUTATIONS; EFFICACY; THERAPY; PATHWAY; SAFETY; FEATURES; 52-WEEK; AGEP; PAIN;

D O I：

10.1111/ddg.14764

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Generalized pustular psoriasis (GPP) is a rare, severe, potentially life-threatening, autoinflammatory, neutrophilic skin disease that may be accompanied by fever and leukocytosis. This paper describes the current state of knowledge on GPP in terms of classification, (differential) diagnosis and prevalence. We present a comparison of the genetics and pathoimmunology of GPP and psoriasis vulgaris with the central mechanisms of autoimmunology and autoinflammation. The currently available therapeutic options, expert recommendations for therapy, and data from early clinical trials investigating targeted therapies will be summarized. We present the results of our discussion with 13 experts for psoriasis vulgaris and GPP and give an integrated overview of indication and therapy based on our personal experience and present an outlook on further research questions. Collectively, this article highlights the high unmet need in GPP, as there exists no satisfactory method of diagnosis or treatment to date and new treatment options will be of great therapeutic benefit to those affected.

引用

页码：753 / 771

页数：19

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